Prion

Prion: A small proteinaceous infectious disease-causing agent that is believed to be the smallest infectious particle. A prion is neither bacterial nor fungal nor viral and contains no genetic material.

Prion Proliferation Models
http://math.la.asu.edu

PRIONS THE INFECTIOUS PROTEINS
http://www.pitt.edu

Transmissible Spongiform Encephalopathies
http://www.life.umd.edu

PRION DISEASES
D. H. Duckworth
http://www.mgm.ufl.edu

Prions (CJD) and Processing of Reusable Medical Products
William A. Rutala, Ph.D., M.P.H.
http://www.unc.edu

Prion Diseases = Transmissible Spongiform Encephalopathies (TSE)
http://www.cst.cmich.edu

Prion  Diseases
http://course1.winona.edu/

What are some  Prion Diseases?
http://www.chem.uwec.edu

A Review of Creutzfeldt-Jakob Disease (CJD) with an Emphasis on Clinical Laboratory Issues
Jeannie Druckenmiller, BS, SM(NRCM), CIC
http://www.slh.wisc.edu

Introduction to Prion Disease and Function
http://phys.columbia.edu

Prion transmission  and the species barrier
http://bigbro.biophys.cornell.edu

Deadly Diseases That Affect Our World
Bridget Sorrell, Bryant Bullard, Charles Prior
ftp://ftp.clarkson.edu

Prions  and Transmissible Spongiform  Encephalopathies
http://chua2.fiu.edu

Prion Disease
http://www.meddean.luc.edu


1000 free full text published articles on Prion

1. The role of activity in synaptic degeneration in a protein misfolding disease, prion disease.
2. Calcium Binding Promotes Prion Protein Fragment 90-231 Conformational Change toward a Membrane Destabilizing and Cytotoxic Structure.
3. Stabilization of a prion strain of synthetic origin requires multiple serial passages.
4. Potential Contribution of Exosomes to the Prion-Like Propagation of Lesions in Alzheimer's Disease.
5. Binding modes of thioflavin T molecules to prion Peptide assemblies identified by using scanning tunneling microscopy.
6. Highly efficient prion transmission by blood transfusion.
7. Correction: Co-Infection with the Friend Retrovirus and Mouse Scrapie Does Not Alter Prion Disease Pathogenesis in Susceptible Mice.
8. Differences in the Curing of [PSI(+)] Prion by Various Methods of Hsp104 Inactivation.
9. Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy Associated with E211K Prion Protein Polymorphism.
10. Mutant p53 aggregates into prion-like amyloid oligomers and fibrils: Implications for cancer.
11. Sequence variations of the bovine prion protein gene (PRNP) in native Korean Hanwoo cattle.
12. Anti-prion activity of brilliant blue g.
13. Gold Nanoparticles as a Probe for Prion Determination via Resonance Light Scattering Method.
14. Protein Disulfide Isomerase Regulates Endoplasmic Reticulum Stress and the Apoptotic Process during Prion Infection and PrP Mutant-Induced Cytotoxicity.
15. Propagation of RML Prions in Mice Expressing PrP Devoid of GPI Anchor Leads to Formation of a Novel, Stable Prion Strain.
16. Incunabular immunological events in prion trafficking.
17. Structural basis for the protective effect of the human prion protein carrying the dominant-negative E219K polymorphism.
18. Multiple substitutions of methionine 129 in human prion protein reveal its importance in the amyloid fibrillation pathway.
19. Common benzothiazole and benzoxazole fluorescent DNA intercalators for studying Alzheimer Aβ1-42 and prion amyloid peptides.
20. The [Het-s] prion, an amyloid fold as a cell death activation trigger.
21. Methionine oxidation perturbs the structural core of the prion protein and suggests a generic misfolding pathway.
22. Sustained translational repression by eIF2α-P mediates prion neurodegeneration.
23. Knockout of the prion protein (PrP)-like Sprn gene does not produce embryonic lethality in combination with PrPC-deficiency.
24. Etymologia: prion.
25. Five questions on prion diseases.
26. Detection of prion protein particles in blood plasma of scrapie infected sheep.
27. Prion formation by a yeast GLFG nucleoporin.
28. Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects.
29. Altered Prion protein expression pattern in CSF as a biomarker for Creutzfeldt-Jakob disease.
30. Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?
31. Prion protein is a key determinant of alcohol sensitivity through the modulation of N-methyl-D-aspartate receptor (NMDAR) activity.
32. Polyglutamine toxicity is controlled by prion composition and gene dosage in yeast.
33. Prion subcellular fractionation reveals infectivity spectrum, with a high titre-low PrPres level disparity.
34. The NALP3 inflammasome is involved in neurotoxic prion peptide-induced microglial activation.
35. Role of the cellular prion protein in oligodendrocyte precursor cell proliferation and differentiation in the developing and adult mouse CNS.
36. Novel prion protein in BSE-affected cattle, Switzerland.
37. The role of cofactors in prion propagation and infectivity.
38. High pressure, a tool to switch between soluble and fibrillar prion protein structures.
39. Early structural features in mammalian prion conformation conversion.
40. Lichens: unexpected anti-prion agents?
41. Phenotypic characterization of cells participating in transport of prion protein aggregates across the intestinal mucosa of sheep.
42. Targeting of prion-infected lymphoid cells to the central nervous system accelerates prion infection.
43. The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells.
44. Molecular pathogenesis of sporadic prion diseases in man.
45. The diverse roles of mononuclear phagocytes in prion disease pathogenesis.
46. The 5' flanking region and intron1 of the bovine prion protein gene (PRNP) are responsible for negative feedback regulation of the prion protein.
47. Allelic origin of protease-sensitive and protease-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease with the P102L mutation.
48. Case for an RNA-prion world: a hypothesis based on conformational diversity.
49. PrionHome: a database of prions and other sequences relevant to prion phenomena.
50. Cellular prion protein expression is not regulated by the Alzheimer's amyloid precursor protein intracellular domain.
51. MicroRNA 146a (miR-146a) is over-expressed during prion disease and modulates the innate immune response and the microglial activation state.
52. A Medicinal Herb Scutellaria lateriflora Inhibits PrP Replication in vitro and Delays the Onset of Prion Disease in Mice.
53. Plasmacytoid dendritic cells sequester high prion titres at early stages of prion infection.
54. Kosmotropic anions promote conversion of recombinant prion protein into a PrPSc-like misfolded form.
55. Biochemical properties of highly neuroinvasive prion strains.
56. Resistance to classical scrapie in experimentally challenged goats carrying mutation K222 of the prion protein gene.
57. Co-infection with the friend retrovirus and mouse scrapie does not alter prion disease pathogenesis in susceptible mice.
58. M cell-depletion blocks oral prion disease pathogenesis.
59. Exacerbation of experimental autoimmune encephalomyelitis in prion protein (PrPc)-null mice: evidence for a critical role of the central nervous system.
60. Neuroanatomical distribution of disease-associated prion protein in experimental bovine spongiform encephalopathy in cattle after intracerebral inoculation.
61. Remarkable reduction of MAP2 in the brains of scrapie-infected rodents and human prion disease possibly correlated with the increase of calpain.
62. Novel prion protein in BSE-affected cattle, Switzerland.
63. Assessing prion infectivity of human urine in sporadic Creutzfeldt-Jakob disease.
64. Multiple effects of the cellular prion protein on tooth development.
65. Non-reducing alkaline solubilization and rapid on-column refolding of recombinant prion protein.
66. A novel expression system for production of soluble prion proteins in E. coli.
67. Prion uptake in the gut: identification of the first uptake and replication sites.
68. An overview of human prion diseases.
69. Assessment of inactivating stop codon mutations in forty Saccharomyces cerevisiae strains: implications for [PSI] prion- mediated phenotypes.
70. Sex effects in mouse prion disease incubation time.
71. Transmission of chronic wasting disease identifies a prion strain causing cachexia and heart infection in hamsters.
72. Isolation of prion with BSE properties from farmed goat.
73. Prion protein promotes growth cone development through reggie/flotillin-dependent N-cadherin trafficking.
74. Genesis of mammalian prions: from non-infectious amyloid fibrils to a transmissible prion disease.
75. Follicular dendritic cell-specific prion protein (PrP) expression alone is sufficient to sustain prion infection in the spleen.
76. The Cellular Prion Protein Prevents Copper-Induced Inhibition of P2X(4) Receptors.
77. Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation.
78. Atypical scrapie isolates involve a uniform prion species with a complex molecular signature.
79. Origins and evolution of the HET-s prion-forming protein: searching for other amyloid-forming solenoids.
80. Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease.
81. Aβ inhibition of ionic conductance in mouse basal forebrain neurons is dependent upon the cellular prion protein PrPC.
82. Lower specific infectivity of protease-resistant prion protein generated in cell-free reactions.
83. Yeast prions assembly and propagation: Contributions of the prion and non-prion moieties and the nature of assemblies.
84. Site-specific structural analysis of a yeast prion strain with species-specific seeding activity.
85. Evidence for retrogene origins of the prion gene family.
86. Dynamic changes and surveillance function of prion protein expression in gastric cancer drug resistance.
87. An overview of animal prion diseases.
88. Development of monoclonal antibodies specific for glycated prion protein.
89. Caprine prion gene polymorphisms are associated with decreased incidence of classical scrapie in goat herds in the United Kingdom.
90. Recruitment of cellular prion protein to mitochondrial raft-like microdomains contributes to apoptosis execution.
91. Instability of the octarepeat region of the human prion protein gene.
92. Oligomeric-induced activity by thienyl pyrimidine compounds traps prion infectivity.
93. PRION-1 scales analysis supports use of functional outcome measures in prion disease.
94. Characterization of spontaneously generated prion-like conformers in cultured cells.
95. Cellular prion protein conformation and function.
96. Pulling rabbits to reveal the secrets of the prion protein.
97. Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
98. The N-terminal, polybasic region is critical for prion protein neuroprotective activity.
99. Inter-allelic prion propagation reveals conformational relationships among a multitude of [PSI] strains.
100. Strain specific resistance to murine scrapie associated with a naturally occurring human prion protein polymorphism at residue 171.
101. High sensitivity of an ELISA kit for detection of the gamma-isoform of 14-3-3 proteins: usefulness in laboratory diagnosis of human prion disease.
102. A nine amino acid domain is essential for mutant prion protein toxicity.
103. Spongiform encephalopathy in transgenic mice expressing a point mutation in the β2-α2 loop of the prion protein.
104. Styryl-based and tricyclic compounds as potential anti-prion agents.
105. Qualitative and quantitative multiplexed proteomic analysis of complex yeast protein fractions that modulate the assembly of the yeast prion Sup35p.
106. Proteinase K-resistant material in ARR/VRQ sheep brain affected with classical scrapie is composed mainly of VRQ prion protein.
107. Expression of prion protein in mouse erythroid progenitors and differentiating murine erythroleukemia cells.
108. Clinical characterization of a kindred with a novel 12-octapeptide repeat insertion in the prion protein gene.
109. Effect of hydrophobic mutations in the H2-H3 subdomain of prion protein on stability and conversion in vitro and in vivo.
110. Classical bovine spongiform encephalopathy by transmission of H-type prion in homologous prion protein context.
111. Can prion disease suspicion be supported earlier? Clinical, radiological and laboratory findings in a series of cases.
112. Comparative analysis of essential collective dynamics and NMR-derived flexibility profiles in evolutionarily diverse prion proteins.
113. Early behavioral changes and quantitative analysis of neuropathological features in murine prion disease: stereological analysis in the albino Swiss mice model.
114. Structural and mechanistic commonalities of amyloid-β and the prion protein.
115. Transcriptomic analysis brings new insight into the biological role of the prion protein during mouse embryogenesis.
116. All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD.
117. Insoluble cellular prion protein and its association with prion and Alzheimer diseases.
118. RNA-binding proteins with prion-like domains in ALS and FTLD-U.
119. Genome wide association studies and prion disease.
120. Cellular prion protein protects from inflammatory and neuropathic pain.
121. Methionine oxidation of Sup35 protein induces formation of the [PSI+] prion in a yeast peroxiredoxin mutant.
122. Calreticulin inhibits prion protein PrP-(23-98) aggregation in vitro.
123. Prion protein in ESC regulation.
124. The sensitive [SWI (+)] prion: new perspectives on yeast prion diversity.
125. Single methyl group determines prion propagation and protein degradation activities of yeast heat shock protein (Hsp)-70 chaperones Ssa1p and Ssa2p.
126. Metal-binding ability of human prion protein fragment peptides analyzed by column switch HPLC.
127. PrP assemblies: spotting the responsible regions in prion propagation.
128. MAVS forms functional prion-like aggregates to activate and propagate antiviral innate immune response.
129. Real-time quaking-induced conversion: a highly sensitive assay for prion detection.
130. Aerosols: an underestimated vehicle for transmission of prion diseases?
131. Allelic frequency and genotypes of prion protein at codon 136 and 171 in Iranian Ghezel sheep breeds.
132. Prion induction by the short-lived, stress-induced protein Lsb2 is regulated by ubiquitination and association with the actin cytoskeleton.
133. Ablation of cellular prion protein does not ameliorate abnormal neural network activity or cognitive dysfunction in the J20 line of human amyloid precursor protein transgenic mice.
134. Physical properties of polymorphic yeast prion amyloid fibers.
135. Neuroanatomical distribution of disease-associated prion protein in cases of bovine spongiform encephalopathy detected by fallen stock surveillance in Japan.
136. Sensitive chemiluminescence detection of prion protein on a membrane by using a peroxidase-labeled dextran probe.
137. Diphenylpyrazole-derived compounds increase survival time of mice after prion infection.
138. The cellular prion protein with a monoacylated glycosylphosphatidylinositol anchor modifies cell membranes, inhibits cell signaling and reduces prion formation.
139. Analysis of nucleic acid chaperoning by the prion protein and its inhibition by oligonucleotides.
140. Conserved properties of human and bovine prion strains on transmission to guinea pigs.
141. Opposing effects of glutamine and asparagine govern prion formation by intrinsically disordered proteins.
142. Prion disease detection, PMCA kinetics, and IgG in urine from sheep naturally/experimentally infected with scrapie and deer with preclinical/clinical chronic wasting disease.
143. Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits.
144. In vivo comparison of chronic wasting disease infectivity from deer with variation at prion protein residue 96.
145. Alteration of the chronic wasting disease species barrier by in vitro prion amplification.
146. Molecular origin of Gerstmann-Sträussler-Scheinker syndrome: insight from computer simulation of an amyloidogenic prion peptide.
147. Pros and cons of a prion-like pathogenesis in Parkinson's disease.
148. Four independent molecular prion protein parameters for discriminating new cases of C, L, and h bovine spongiform encephalopathy in cattle.
149. Production of a recombinant full-length prion protein in a soluble form without refolding or detergents.
150. A small, glutamine-free domain propagates the [SWI(+)] prion in budding yeast.
151. Interaction between prion protein and toxic amyloid β assemblies can be therapeutically targeted at multiple sites.
152. Insights into prion biology: integrating a protein misfolding pathway with its cellular environment.
153. Unaltered prion protein expression in Alzheimer disease patients.
154. Photodegradation illuminates the role of polyanions in prion infectivity.
155. Neuroprotective effects of the cellular prion protein in autoimmune optic neuritis.
156. Amyloid of the Candida albicans Ure2p prion domain is infectious and has an in-register parallel β-sheet structure.
157. Gene expression profiling and association with prion-related lesions in the medulla oblongata of symptomatic natural scrapie animals.
158. Prion protein function and the disturbance of early embryonic development in zebrafish.
159. Duration of prion disease is longer in Japan than in other countries.
160. Retraction for Nemecek et al.: A prion of yeast metacaspase homolog (Mca1p) detected by a genetic screen.
161. Prion formation and polyglutamine aggregation are controlled by two classes of genes.
162. A multiscale approach to characterize the early aggregation steps of the amyloid-forming peptide GNNQQNY from the yeast prion sup-35.
163. PrP(Sc)-specific antibodies with the ability to immunodetect prion oligomers.
164. Structural and functional neuroimaging in human prion diseases.
165. Lack of a-disintegrin-and-metalloproteinase ADAM10 leads to intracellular accumulation and loss of shedding of the cellular prion protein in vivo.
166. Inherited prion disease with 4-octapeptide repeat insertion: disease requires the interaction of multiple genetic risk factors.
167. Enhanced neural progenitor/stem cells self-renewal via the interaction of stress-inducible protein 1 with the prion protein.
168. Modeling routes of chronic wasting disease transmission: environmental prion persistence promotes deer population decline and extinction.
169. Structural underpinnings of prion protein conversion.
170. Alzheimer's disease brain-derived amyloid-β-mediated inhibition of LTP in vivo is prevented by immunotargeting cellular prion protein.
171. A new colour assay for [URE3] prion in a genetic background used to score for the [PSI⁺] prion.
172. Degradation of the disease-associated prion protein by a serine protease from lichens.
173. Prion disease blood test using immunoprecipitation and improved quaking-induced conversion.
174. Cyclosporin-A-induced prion protein aggresomes are dynamic quality-control cellular compartments.
175. Polymorphisms of the prion protein gene and their effects on litter size and risk evaluation for scrapie in Chinese Hu sheep.
176. Disulfide mapping reveals the domain swapping as the crucial process of the structural conversion of prion protein.
177. Functions of yeast Hsp40 chaperone Sis1p dispensable for prion propagation but important for prion curing and protection from prion toxicity.
178. Dynamic diagnosis of familial prion diseases supports the β2-α2 loop as a universal interference target.
179. Effect of glycans and the glycophosphatidylinositol anchor on strain dependent conformations of scrapie prion protein: improved purifications and infrared spectra.
180. Effects of solution chemistry and aging time on prion protein adsorption and replication of soil-bound prions.
181. Detection of chronic wasting disease prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission.
182. Rapid cell-surface prion protein conversion revealed using a novel cell system.
183. The core of Ure2p prion fibrils is formed by the N-terminal segment in a parallel cross-β structure: evidence from solid-state NMR.
184. Presence and seeding activity of pathological prion protein (PrP(TSE)) in skeletal muscles of white-tailed deer infected with chronic wasting disease.
185. Prion protein expression regulates embryonic stem cell pluripotency and differentiation.
186. An overlapping reading frame in the PRNP gene encodes a novel polypeptide distinct from the prion protein.
187. Self-propagation and transmission of misfolded mutant SOD1: prion or prion-like phenomenon?
188. A quantitative proteomic approach to prion disease biomarker research: delving into the glycoproteome.
189. Molecular chaperone Hsp104 can promote yeast prion generation.
190. In vitro amplification of misfolded prion protein using lysate of cultured cells.
191. Investigation of the effects of experimental autolysis on the detection of abnormal prion protein in lymphoid and central nervous system tissues from elk and sheep using the Western blotting method.
192. Molecular basis for transmission barrier and interference between closely related prion proteins in yeast.
193. Distinct proteinase K-resistant prion protein fragment in goats with no signs of disease in a classical scrapie outbreak.
194. Detection of prion protein in urine-derived injectable fertility products by a targeted proteomic approach.
195. Prion protein polymorphisms affect chronic wasting disease progression.
196. The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication.
197. Btn3 is a negative regulator of Btn2-mediated endosomal protein trafficking and prion curing in yeast.
198. Dominant prion mutants induce curing through pathways that promote chaperone-mediated disaggregation.
199. Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype.
200. PrP-specific camel antibodies with the ability to immunodetect intracellular prion protein.
201. Decreased CSF transferrin in sCJD: a potential pre-mortem diagnostic test for prion disorders.
202. Developmental influence of the cellular prion protein on the gene expression profile in mouse hippocampus.
203. Suicidal [PSI+] is a lethal yeast prion.
204. Infrared Microspectroscopy: A Multiple-Screening Platform for Investigating Single-Cell Biochemical Perturbations upon Prion Infection.
205. Amyloid-beta42 interacts mainly with insoluble prion protein in the Alzheimer brain.
206. Destabilization and recovery of a yeast prion after mild heat shock.
207. An N-terminal polybasic domain and cell surface localization are required for mutant prion protein toxicity.
208. [SWI], the prion formed by the chromatin remodeling factor Swi1, is highly sensitive to alterations in Hsp70 chaperone system activity.
209. Prion-forming ability of Ure2 of yeasts is not evolutionarily conserved.
210. Amyloid features and neuronal toxicity of mature prion fibrils are highly sensitive to high pressure.
211. Superoxide generation catalyzed by the ozone-inducible plant peptides analogous to prion octarepeat motif.
212. Prion diseases of yeast: amyloid structure and biology.
213. Normal modes of prion proteins: from native to infectious particle.
214. Radically different amyloid conformations dictate the seeding specificity of a chimeric Sup35 prion.
215. Soil clay content underlies prion infection odds.
216. Globular domain of the prion protein needs to be unlocked by domain swapping to support prion protein conversion.
217. PrPSc spreading patterns in the brain of sheep linked to different prion types.
218. Effects of polymorphisms in ovine and caprine prion protein alleles on cell-free conversion.
219. Atomic structures suggest determinants of transmission barriers in mammalian prion disease.
220. Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells.
221. In situ photodegradation of incorporated polyanion does not alter prion infectivity.
222. HEPES inhibits the conversion of prion protein in cell culture.
223. The suppression of prion propagation using poly-L-lysine by targeting plasminogen that stimulates prion protein conversion.
224. Specific binding of the pathogenic prion isoform: development and characterization of a humanized single-chain variable antibody fragment.
225. Emergence of multiple prion strains from single isolates of ovine scrapie.
226. The role of predation in disease control: a comparison of selective and nonselective removal on prion disease dynamics in deer.
227. Spin hamiltonian parameters for Cu(II)-prion peptide complexes from L-band electron paramagnetic resonance spectroscopy.
228. Conserved stress-protective activity between prion protein and Shadoo.
229. One octarepeate expansion to the human prion protein alters both the Zn2+ and Cu2+ coordination environments within the octarepeate domain.
230. Prion propagation in cells expressing PrP glycosylation mutants.
231. 2-Aminothiazoles as therapeutic leads for prion diseases.
232. Relationship between prion propensity and the rates of individual molecular steps of fibril assembly.
233. Glycosylphosphatidylinositol anchor-dependent stimulation pathway required for generation of baculovirus-derived recombinant scrapie prion protein.
234. Extraneural manifestations of prion infection in GPI-anchorless transgenic mice.
235. In Sup35p filaments (the [PSI+] prion), the globular C-terminal domains are widely offset from the amyloid fibril backbone.
236. Meat and bone meal and mineral feed additives may increase the risk of oral prion disease transmission.
237. Monoacylated cellular prion protein modifies cell membranes, inhibits cell signaling, and reduces prion formation.
238. Recovery of small infectious PrP(res) aggregates from prion-infected cultured cells.
239. Prion protein misfolding affects calcium homeostasis and sensitizes cells to endoplasmic reticulum stress.
240. Molecular typing of protease-resistant prion protein in transmissible spongiform encephalopathies of small ruminants, France, 2002-2009.
241. Ramanujan Hegde: The prion puzzle and protein translocation. Interview by Caitlin Sedwick.
242. Conversion of bacterially expressed recombinant prion protein.
243. Signal transduction in neurons: effects of cellular prion protein on fyn kinase and ERK1/2 kinase.
244. Neuroimaging in human prion disease: Searching in the mist.
245. Transfer of a prion strain to different hosts leads to emergence of strain variants.
246. Conditional modulation of membrane protein expression in cultured cells mediated by prion protein recognition of short phosphorothioate oligodeoxynucleotides.
247. Evidence for varied aetiologies regulating the transmission of prion disease: implications for understanding the heritable basis of prion incubation times.
248. Rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays.
249. The retinoic acid receptor beta (Rarb) region of Mmu14 is associated with prion disease incubation time in mouse.
250. Molecular interactions between prions as seeds and recombinant prion proteins as substrates resemble the biological interspecies barrier in vitro.
251. β-amyloid oligomers and prion protein: Fatal attraction?
252. Alimentary prion infections: Touchdown in the intestine.
253. The yeast prion case: could there be a uniform concept underlying complex protein folding?
254. Implications of the prion-related Q/N domains in TDP-43 and FUS.
255. Prion hypothesis: the end of the controversy?
256. Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob diseased brain using conformation-dependent immunoassay and Western blot.
257. Crucial role for prion protein membrane anchoring in the neuroinvasion and neural spread of prion infection.
258. Reticulon 3 attenuates the clearance of cytosolic prion aggregates via inhibiting autophagy.
259. A Drug-Based Cellular Assay (DBCA) for studying cytotoxic and cytoprotective activities of the prion protein: A practical guide.
260. Optical trapping with high forces reveals unexpected behaviors of prion fibrils.
261. Pathologic evidence that the T188R mutation in PRNP is associated with prion disease.
262. Immunomodulation for prion and prion-related diseases.
263. Intraspecies prion transmission results in selection of sheep scrapie strains.
264. Prion and TNFα: TAC(E)it agreement between the prion protein and cell signaling.
265. Dry amyloid fibril assembly in a yeast prion peptide is mediated by long-lived structures containing water wires.
266. Flexibility of the Ure2 prion domain is important for amyloid fibril formation.
267. Rapidly progressive dementias and the treatment of human prion diseases.
268. Preclinical deposition of pathological prion protein in muscle of experimentally infected primates.
269. Combination of the somatic cell nuclear transfer method and RNAi technology for the production of a prion gene-knockdown calf using plasmid vectors harboring the U6 or tRNA promoter.
270. Prion protein in Caenorhabditis elegans: Distinct models of anti-BAX and neuropathology.
271. A standardized comparison of commercially available prion decontamination reagents using the Standard Steel-Binding Assay.
272. The prion hypothesis: from biological anomaly to basic regulatory mechanism.
273. Near-infrared fluorescence imaging of apoptotic neuronal cell death in a live animal model of prion disease.
274. Deletion of a Ure2 C-terminal prion-inhibiting region promotes the rate of fibril seed formation and alters interaction with Hsp40.
275. Cell type-specific neuroprotective activity of untranslocated prion protein.
276. Molecular biology and pathology of prion strains in sporadic human prion diseases.
277. Early embryonic gene expression profiling of zebrafish prion protein (Prp2) morphants.
278. Prion disease susceptibility is affected by beta-structure folding propensity and local side-chain interactions in PrP.
279. A size threshold limits prion transmission and establishes phenotypic diversity.
280. Compartment-restricted biotinylation reveals novel features of prion protein metabolism in vivo.
281. A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report.
282. Identification and structural analysis of C-terminally truncated collapsin response mediator protein-2 in a murine model of prion diseases.
283. Prion replication in the hematopoietic compartment is not required for neuroinvasion in scrapie mouse model.
284. Diverse effects on the native β-sheet of the human prion protein due to disease-associated mutations.
285. Solution structure and dynamics of the I214V mutant of the rabbit prion protein.
286. Calcineurin inhibition at the clinical phase of prion disease reduces neurodegeneration, improves behavioral alterations and increases animal survival.
287. Striatal pathology underlies prion infection-mediated hyperactivity in mice.
288. Nucleic acid-free mutation of prion strains.
289. Prion transmission: prion excretion and occurrence in the environment.
290. Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding.
291. Proteomic consequences of expression and pathological conversion of the prion protein in inducible neuroblastoma N2a cells.
292. Expression and knockdown of cellular prion protein (PrPC) in differentiating mouse embryonic stem cells.
293. Influence of pH on the human prion protein: insights into the early steps of misfolding.
294. Quantitative phosphoproteomic analysis of prion-infected neuronal cells.
295. The intricate mechanisms of neurodegeneration in prion diseases.
296. Prion genotypes of scrapie-infected Canadian sheep 1998-2008.
297. Magnetization transfer ratio may be a surrogate of spongiform change in human prion diseases.
298. Review: contribution of transgenic models to understanding human prion disease.
299. Loss of Octarepeats in two processed prion pseudogenes in the red squirrel, Sciurus vulgaris.
300. Metabotropic glutamate receptors transduce signals for neurite outgrowth after binding of the prion protein to laminin γ1 chain.
301. Pharmacological chaperone for the structured domain of human prion protein.
302. Structural requirements for efficient prion protein conversion: cofactors may promote a conversion-competent structure for PrP(C).
303. Prospective 10-year surveillance of human prion diseases in Japan.
304. Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay.
305. Engulfment of cerebral apoptotic bodies controls the course of prion disease in a mouse strain-dependent manner.
306. Role of alpha7 nicotinic acetylcholine receptor in calcium signaling induced by prion protein interaction with stress-inducible protein 1.
307. Transport of the pathogenic prion protein through soils.
308. Prion strain interactions are highly selective.
309. Digestion and transportation of bovine spongiform encephalopathy-derived prion protein in the sheep intestine.
310. Amyloid-like aggregates of the yeast prion protein ure2 enter vertebrate cells by specific endocytotic pathways and induce apoptosis.
311. Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease.
312. Increased expression and local accumulation of the prion protein, Alzheimer Aβ peptides, superoxide dismutase 1, and nitric oxide synthases 1 & 2 in muscle in a rabbit model of diabetes.
313. Sequence-dependent prion protein misfolding and neurotoxicity.
314. Glycosaminoglycan sulphation affects the seeded misfolding of a mutant prion protein.
315. Epitope-specific anti-prion antibodies upregulate apolipoprotein E and disrupt membrane cholesterol homeostasis.
316. Plasminogen stimulates propagation of protease-resistant prion protein in vitro.
317. Prion amyloid structure explains templating: how proteins can be genes.
318. Nanoimaging for prion related diseases.
319. PrPC, the cellular isoform of the human prion protein, is a novel biomarker of HIV-associated neurocognitive impairment and mediates neuroinflammation.
320. Role of the highly conserved middle region of prion protein (PrP) in PrP-lipid interaction.
321. A yeast toxic mutant of HET-s((218-289)) prion displays alternative intermediates of amyloidogenesis.
322. The prion protein as a receptor for amyloid-beta.
323. Prion protein in Alzheimer's pathogenesis: a hot and controversial issue.
324. Binding of pro-prion to filamin A: by design or an unfortunate blunder.
325. Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein.
326. Human prion strain selection in transgenic mice.
327. Molecular pathology of human prion disease.
328. Proteasome activity and biological properties of normal prion protein: a comparison between young and aged cattle.
329. Crystallographic studies of prion protein (PrP) segments suggest how structural changes encoded by polymorphism at residue 129 modulate susceptibility to human prion disease.
330. Is the presence of abnormal prion protein in the renal glomeruli of feline species presenting with FSE authentic?
331. Distinct subregions of Swi1 manifest striking differences in prion transmission and SWI/SNF function.
332. Cellular prion protein promotes regeneration of adult muscle tissue.
333. Anionic phospholipid interactions of the prion protein N terminus are minimally perturbing and not driven solely by the octapeptide repeat domain.
334. Probing the conformation of a prion protein fibril with hydrogen exchange.
335. Characterization of the prion protein in human urine.
336. Characterization of the role of dendritic cells in prion transfer to primary neurons.
337. Prion protein and Abeta-related synaptic toxicity impairment.
338. NMR structure of the human prion protein with the pathological Q212P mutation reveals unique structural features.
339. Neuroinvasion in prion diseases: the roles of ascending neural infection and blood dissemination.
340. Morphological and functional abnormalities in mitochondria associated with synaptic degeneration in prion disease.
341. Pro-prion binds filamin A, facilitating its interaction with integrin beta1, and contributes to melanomagenesis.
342. Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse.
343. Unique structural characteristics of the rabbit prion protein.
344. The hydrophobic core region governs mutant prion protein aggregation and intracellular retention.
345. Paradoxical role of prion protein aggregates in redox-iron induced toxicity.
346. Cell-based immunotherapy of prion diseases by adoptive transfer of antigen-loaded dendritic cells or antigen-primed CD(4+) T lymphocytes.
347. [Prion diseases: what is the role of dendritic cells in the pathogenesis of transmissible prion diseases?].
348. Prion protein interaction with stress-inducible protein 1 enhances neuronal protein synthesis via mTOR.
349. Cellular form of prion protein inhibits Reelin-mediated shedding of Caspr from the neuronal cell surface to potentiate Caspr-mediated inhibition of neurite outgrowth.
350. Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein.
351. [Cellular prion protein in the central nervous system of mammals. Anatomoclinical associations].
352. Immunohistochemical detection of disease-associated prion protein in the intestine of cattle naturally affected with bovine spongiform encephalopathy by using an alkaline-based chemical antigen retrieval method.
353. Sex effect in mouse and human prion disease.
354. Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role OF N-terminal residues.
355. Neurotoxic mutants of the prion protein induce spontaneous ionic currents in cultured cells.
356. Two amyloid States of the prion protein display significantly different folding patterns.
357. A molecular switch controls interspecies prion disease transmission in mice.
358. The fatal attraction between pro-prion and filamin A: prion as a marker in human cancers.
359. Aptamer-mediated chemiluminescence detection of prion protein on a membrane using trimethoxyphenylglyoxal.
360. Conformational stability of Syrian hamster prion protein PrP(90-231).
361. Conformation-dependent high-affinity monoclonal antibodies to prion proteins.
362. Faecal CWD prion excretion and inflammation.
363. Proliferative arrest of neural cells induces prion protein synthesis, nanotube formation, and cell-to-cell contacts.
364. Non-Mendelian determinant [ISP+] in yeast is a nuclear-residing prion form of the global transcriptional regulator Sfp1.
365. The effects of amino acid composition on yeast prion formation and prion domain interactions.
366. Conversion of a yeast prion protein to an infectious form in bacteria.
367. A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death.
368. Prion strain mutation determined by prion protein conformational compatibility and primary structure.
369. Association of N176K and L141F dimorphisms of the PRNP gene with lack of pathological prion protein deposition in placentas of naturally and experimentally scrapie-affected ARQ/ARQ sheep.
370. Cellular prion protein released on exosomes from macrophages binds to Hsp70.
371. Reiterating the epitope specificity of prion-specific mAb 3F4.
372. Memory impairment in transgenic Alzheimer mice requires cellular prion protein.
373. Genetic and epigenetic control of the efficiency and fidelity of cross-species prion transmission.
374. Analysis of the [RNQ+] prion reveals stability of amyloid fibers as the key determinant of yeast prion variant propagation.
375. Experimental oral transmission of chronic wasting disease to red deer (Cervus elaphus elaphus): early detection and late stage distribution of protease-resistant prion protein.
376. Ovine reference materials and assays for prion genetic testing.
377. Combined diffusion imaging and MR spectroscopy in the diagnosis of human prion diseases.
378. Residues surrounding the glycosylphosphatidylinositol anchor attachment site of PrP modulate prion infection: insight from the resistance of rabbits to prion disease.
379. Glycosylphosphatidylinositol anchor analogues sequester cholesterol and reduce prion formation.
380. Prion induction involves an ancient system for the sequestration of aggregated proteins and heritable changes in prion fragmentation.
381. The physical relationship between infectivity and prion protein aggregates is strain-dependent.
382. Prion shedding from olfactory neurons into nasal secretions.
383. Cellular factors implicated in prion replication.
384. The oligomerization properties of prion protein are restricted to the H2H3 domain.
385. Complement protein C1q forms a complex with cytotoxic prion protein oligomers.
386. A kiss of a prion: new implications for oral transmissibility.
387. Neuron dysfunction is induced by prion protein with an insertional mutation via a Fyn kinase and reversed by sirtuin activation in Caenorhabditis elegans.
388. Detection of the abnormal isoform of the prion protein associated with chronic wasting disease in the optic pathways of the brain and retina of Rocky Mountain elk (Cervus elaphus nelsoni).
389. Species-dependent differences in cofactor utilization for formation of the protease-resistant prion protein in vitro.
390. PrP-specific camel antibodies with the ability to immunodetect intracellular prion protein.
391. Prion fibrillization is mediated by a native structural element that comprises helices H2 and H3.
392. Phospholipase A2 inhibitors protect against prion and Abeta mediated synapse degeneration.
393. Extra N-terminal residues have a profound effect on the aggregation properties of the potential yeast prion protein Mca1.
394. Molecular cross talk between misfolded proteins in animal models of Alzheimer's and prion diseases.
395. Prion-like disorders: blurring the divide between transmissibility and infectivity.
396. Conservation of a glycine-rich region in the prion protein is required for uptake of prion infectivity.
397. MicroRNAs as a molecular basis for mental retardation, Alzheimer's and prion diseases.
398. Structure and assembly properties of the N-terminal domain of the prion Ure2p in isolation and in its natural context.
399. A camelid anti-PrP antibody abrogates PrP replication in prion-permissive neuroblastoma cell lines.
400. Characterizing the assembly of the Sup35 yeast prion fragment, GNNQQNY: structural changes accompany a fiber-to-crystal switch.
401. Changing the solvent accessibility of the prion protein disulfide bond markedly influences its trafficking and effect on cell function.
402. Prion-like transmission of protein aggregates in neurodegenerative diseases.
403. Ribosome-associated peroxiredoxins suppress oxidative stress-induced de novo formation of the [PSI+] prion in yeast.
404. Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors.
405. Genetic Prion Diseases.
406. Coinfecting prion strains compete for a limiting cellular resource.
407. Application of "omics" to prion biomarker discovery.
408. Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein membrane anchoring.
409. B cells and platelets harbor prion infectivity in the blood of deer infected with chronic wasting disease.
410. [Acquired human prion diseases--past and present issues].
411. PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
412. A novel seven-octapeptide repeat insertion in the prion protein gene (PRNP) in a Dutch pedigree with Gerstmann-Sträussler-Scheinker disease phenotype: comparison with similar cases from the literature.
413. The glycosylphosphatidylinositol anchor is a major determinant of prion binding and replication.
414. The octarepeat region of the prion protein is conformationally altered in PrP(Sc).
415. Brain-water diffusion coefficients reflect the severity of inherited prion disease.
416. Tracing conformational transition of abnormal prion proteins during interspecies transmission by using novel antibodies.
417. Manganese upregulates cellular prion protein and contributes to altered stabilization and proteolysis: relevance to role of metals in pathogenesis of prion disease.
418. Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations.
419. The Prion Disease Database: a comprehensive transcriptome resource for systems biology research in prion diseases.
420. Signal sequence insufficiency contributes to neurodegeneration caused by transmembrane prion protein.
421. Endogenous proteolytic cleavage of disease-associated prion protein to produce C2 fragments is strongly cell- and tissue-dependent.
422. Pharmacological prion protein silencing accelerates central nervous system autoimmune disease via T cell receptor signalling.
423. Fragment molecular orbital calculations reveal that the E200K mutation markedly alters local structural stability in the human prion protein.
424. Synthetic amyloid-beta oligomers impair long-term memory independently of cellular prion protein.
425. Conformational flexibility of Y145Stop human prion protein amyloid fibrils probed by solid-state nuclear magnetic resonance spectroscopy.
426. Insights into prion protein function from atomistic simulations.
427. Generating a prion with bacterially expressed recombinant prion protein.
428. Distinct type of transmission barrier revealed by study of multiple prion determinants of Rnq1.
429. Low density subcellular fractions enhance disease-specific prion protein misfolding.
430. Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
431. Experimental verification of a traceback phenomenon in prion infection.
432. Exploring prion protein biology in flies: genetics and beyond.
433. Differences in prion strain conformations result from non-native interactions in a nucleus.
434. Neuroprotective function of cellular prion protein in a mouse model of amyotrophic lateral sclerosis.
435. Refinement of under-determined loops of Human Prion Protein by database-derived distance constraints.
436. Recombinant prion protein induces a new transmissible prion disease in wild-type animals.
437. Human prion diseases in the United States.
438. Neuronal low-density lipoprotein receptor-related protein 1 binds and endocytoses prion fibrils via receptor cluster 4.
439. Distinct molecular signature of bovine spongiform encephalopathy prion in pigs.
440. Glimepiride reduces the expression of PrPc, prevents PrPSc formation and protects against prion mediated neurotoxicity in cell lines.
441. Localization of prion-destabilizing mutations in the N-terminal non-prion domain of Rnq1 in Saccharomyces cerevisiae.
442. Mutant prion protein expression is associated with an alteration of the Rab GDP dissociation inhibitor alpha (GDI)/Rab11 pathway.
443. Molecular model of prion transmission to humans.
444. Beta-amyloid oligomers and cellular prion protein in Alzheimer's disease.
445. Implications of conflicting associations of the prion protein (PrP) gene with scrapie susceptibility and fitness on the persistence of scrapie.
446. Chemical induction of misfolded prion protein conformers in cell culture.
447. Prion protein self-peptides modulate prion interactions and conversion.
448. A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein.
449. Glypican-1 mediates both prion protein lipid raft association and disease isoform formation.
450. Conformational diversity in prion protein variants influences intermolecular beta-sheet formation.
451. Amyloid fibrils of human prion protein are spun and woven from morphologically disordered aggregates.
452. A novel protective prion protein variant that colocalizes with kuru exposure.
453. The spontaneous appearance rate of the yeast prion [PSI+] and its implications for the evolution of the evolvability properties of the [PSI+] system.
454. Immunopurification of pathological prion protein aggregates.
455. A synergistic small-molecule combination directly eradicates diverse prion strain structures.
456. Design and construction of diverse mammalian prion strains.
457. Structure of the flexible amino-terminal domain of prion protein bound to a sulfated glycan.
458. Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP.
459. The cellular prion protein identifies bipotential cardiomyogenic progenitors.
460. Context dependent neuroprotective properties of prion protein (PrP).
461. Prion-like propagation of cytosolic protein aggregates: insights from cell culture models.
462. Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease.
463. Cholesterol secosterol adduction inhibits the misfolding of a mutant prion protein fragment that induces neurodegeneration.
464. Prediction of prion protein genotype and association of this genotype with lamb performance traits of Suffolk sheep.
465. The Hofmeister effect on amyloid formation using yeast prion protein.
466. Is, indeed, the prion protein a Harlequin servant of "many" masters?
467. Prion neurodegeneration: starts and stops at the synapse.
468. Prion protein and Alzheimer disease.
469. De novo mammalian prion synthesis.
470. Compositional determinants of prion formation in yeast.
471. Isolation of two distinct prion strains from a scrapie-affected sheep.
472. Mouse vaccination with dendritic cells loaded with prion protein peptides overcomes tolerance and delays scrapie.
473. Mountain lions prey selectively on prion-infected mule deer.
474. Prion dynamics and the quest for the genetic determinant in protein-only inheritance.
475. Characterization of the amyloid bacterial inclusion bodies of the HET-s fungal prion.
476. Prion protein misfolding.
477. Regulation of GABA(A) and glutamate receptor expression, synaptic facilitation and long-term potentiation in the hippocampus of prion mutant mice.
478. The role of glycophosphatidylinositol anchor in the amplification of the scrapie isoform of prion protein in vitro.
479. The alpha-secretase-derived N-terminal product of cellular prion, N1, displays neuroprotective function in vitro and in vivo.
480. Similarities between forms of sheep scrapie and Creutzfeldt-Jakob disease are encoded by distinct prion types.
481. Adoptive transfer of T lymphocytes sensitized against the prion protein attenuates prion invasion in scrapie-infected mice.
482. Manganese enhances prion protein survival in model soils and increases prion infectivity to cells.
483. Clathrin-independent internalization of normal cellular prion protein in neuroblastoma cells is associated with the Arf6 pathway.
484. Human Prion disease with a T188K mutation in Chinese: a case report.
485. Strain-specific proteolytic processing of the prion protein in prion diseases of ruminants transmitted in ovine transgenic mice.
486. Inhibition of RNA recruitment and replication of an RNA virus by acridine derivatives with known anti-prion activities.
487. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.
488. Ligand binding and hydration in protein misfolding: insights from studies of prion and p53 tumor suppressor proteins.
489. Cell density-dependent increase in the level of protease-resistant prion protein in prion-infected Neuro2a mouse neuroblastoma cells.
490. Antimicrobial activity of human prion protein is mediated by its N-terminal region.
491. Transmissibility of atypical scrapie in ovine transgenic mice: major effects of host prion protein expression and donor prion genotype.
492. Quantitative and qualitative analysis of cellular prion protein (PrP(C)) expression in bovine somatic tissues.
493. Natural and synthetic prion structure from X-ray fiber diffraction.
494. Redox control of prion and disease pathogenesis.
495. A heritable switch in carbon source utilization driven by an unusual yeast prion.
496. A Copine family member, Cpne8, is a candidate quantitative trait gene for prion disease incubation time in mouse.
497. The role of the prion protein membrane anchor in prion infection.
498. Evolutionary descent of prion genes from the ZIP family of metal ion transporters.
499. Degenerating synaptic boutons in prion disease: microglia activation without synaptic stripping.
500. Requirements of Hsp104p activity and Sis1p binding for propagation of the [RNQ(+)] prion.
501. Targeting of the prion protein to the cytosol: mechanisms and consequences.
502. Prion protein and metal interaction: physiological and pathological implications.
503. Autophagy, prion infection and their mutual interactions.
504. Prion protein: orchestrating neurotrophic activities.
505. Prion neurotoxicity: insights from prion protein mutants.
506. The prion protein: friend and foe.
507. The Schizosaccharomyces pombe Hsp104 disaggregase is unable to propagate the [PSI] prion.
508. Magnetic resonance diagnostic markers in clinically sporadic prion disease: a combined brain magnetic resonance imaging and spectroscopy study.
509. A promiscuous prion: efficient induction of [URE3] prion formation by heterologous prion domains.
510. Transgenic mice expressing porcine prion protein resistant to classical scrapie but susceptible to sheep bovine spongiform encephalopathy and atypical scrapie.
511. Prion-induced activation of cholesterogenic gene expression by Srebp2 in neuronal cells.
512. Functionally relevant domains of the prion protein identified in vivo.
513. Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.
514. Upregulation of miRNA hsa-miR-342-3p in experimental and idiopathic prion disease.
515. Aggregation and amyloid fibril formation induced by chemical dimerization of recombinant prion protein in physiological-like conditions.
516. Analysis of protein levels of 24 cytokines in scrapie agent-infected brain and glial cell cultures from mice differing in prion protein expression levels.
517. Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice.
518. Influence of prion strain on prion protein adsorption to soil in a competitive matrix.
519. Reciprocal remodeling upon binding of the prion protein to its signaling partner hop/STI1.
520. Complex polyamines: unique prion disaggregating compounds.
521. Prion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes.
522. Mutations at codons 178, 200-129, and 232 contributed to the inherited prion diseases in Korean patients.
523. Impaired axonal transport in motor neurons correlates with clinical prion disease.
524. Differential stability of the bovine prion protein upon urea unfolding.
525. Binding of pro-prion to filamin A disrupts cytoskeleton and correlates with poor prognosis in pancreatic cancer.
526. Allelic discrimination of genetic human prion diseases by real-time PCR genotyping.
527. Sequestration of essential proteins causes prion associated toxicity in yeast.
528. Loss of anti-Bax function in Gerstmann-Sträussler-Scheinker syndrome-associated prion protein mutants.
529. Function of SSA subfamily of Hsp70 within and across species varies widely in complementing Saccharomyces cerevisiae cell growth and prion propagation.
530. Unique quadruplex structure and interaction of an RNA aptamer against bovine prion protein.
531. Is Parkinson's disease a prion disorder?
532. Enhancement of protein misfolding cyclic amplification by using concentrated cellular prion protein source.
533. The cellular prion protein interacts with the tissue non-specific alkaline phosphatase in membrane microdomains of bioaminergic neuronal cells.
534. Trans-dominant inhibition of prion propagation in vitro is not mediated by an accessory cofactor.
535. Cytosolic prion protein induces apoptosis in human neuronal cell SH-SY5Y via mitochondrial disruption pathway.
536. Prion protein expression and release by mast cells after activation.
537. Prion proteins with pathogenic and protective mutations show similar structure and dynamics.
538. Prion adsorption to stainless steel is promoted by nickel and molybdenum.
539. The consequences of pathogenic mutations to the human prion protein.
540. Therapy for prion diseases: Insights from the use of RNA interference.
541. Doxorubicin and congo red effectiveness on prion infectivity in golden Syrian hamster.
542. Distinct structures of scrapie prion protein (PrPSc)-seeded versus spontaneous recombinant prion protein fibrils revealed by hydrogen/deuterium exchange.
543. Phosphorylation of prion protein at serine 43 induces prion protein conformational change.
544. Ultraviolet-ozone treatment reduces levels of disease-associated prion protein and prion infectivity.
545. Investigating the conformational stability of prion strains through a kinetic replication model.
546. Prion infectivity in fat of deer with chronic wasting disease.
547. Human variant Creutzfeldt-Jakob disease and sheep scrapie PrP(res) detection using seeded conversion of recombinant prion protein.
548. Prion protein (PrP) knock-out mice show altered iron metabolism: a functional role for PrP in iron uptake and transport.
549. Genomic and post-genomic analyses of human prion diseases.
550. Role of ADAMs in the ectodomain shedding and conformational conversion of the prion protein.
551. Functional implications of multistage copper binding to the prion protein.
552. Glycosylation modification of human prion protein provokes apoptosis in HeLa cells in vitro.
553. The cellular prion protein and its role in Alzheimer disease.
554. Influence of Hsp70s and their regulators on yeast prion propagation.
555. Prion protein-detergent micelle interactions studied by NMR in solution.
556. Selective processing and metabolism of disease-causing mutant prion proteins.
557. Copper binding extrinsic to the octarepeat region in the prion protein.
558. Prion protein paralog doppel protein interacts with alpha-2-macroglobulin: a plausible mechanism for doppel-mediated neurodegeneration.
559. Prion propagation by Hsp40 molecular chaperones.
560. Comparative prion disease gene expression profiling using the prion disease mimetic, cuprizone.
561. Functional depletion of mahogunin by cytosolically exposed prion protein contributes to neurodegeneration.
562. Spongiform pathology in mouse CNS lacking 'neuropathy target esterase' and cellular prion protein.
563. Limited transcriptional response of ovine microglia to prion accumulation.
564. Prion variants, species barriers, generation and propagation.
565. Hsp104 and prion propagation.
566. Shadoo (Sprn) and prion disease incubation time in mice.
567. In vivo generation of neurotoxic prion protein: role for hsp70 in accumulation of misfolded isoforms.
568. Prion protein expression and processing in human mononuclear cells: the impact of the codon 129 prion gene polymorphism.
569. Effects of lipid composition and phase on the membrane interaction of the prion peptide 106-126 amide.
570. Prion strain discrimination based on rapid in vivo amplification and analysis by the cell panel assay.
571. Tunnelling nanotubes: a highway for prion spreading?
572. Heterologous cross-seeding mimics cross-species prion conversion in a yeast model.
573. Biochemical typing of pathological prion protein in aging cattle with BSE.
574. Genetic variability of the prion protein gene (PRNP) in wild ruminants from Italy and Scotland.
575. Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey.
576. p53-Dependent transcriptional control of cellular prion by presenilins.
577. Alternative translation initiation generates cytoplasmic sheep prion protein.
578. The expanding realm of prion phenomena in neurodegenerative disease.
579. Prion protein biosynthesis and its emerging role in neurodegeneration.
580. Chemical and biophysical insights into the propagation of prion strains.
581. Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease.
582. Retinal function and morphology are altered in cattle infected with the prion disease transmissible mink encephalopathy.
583. Identification of an intracellular site of prion conversion.
584. Transport of prion protein across the blood-brain barrier.
585. Thermo-stable nature of aromatic monoamine-dependent superoxide-generating activity of human prion-derived Cu-binding peptides.
586. Design of anti- and pro-aggregation variants to assess the effects of methionine oxidation in human prion protein.
587. Increased [PSI+] appearance by fusion of Rnq1 with the prion domain of Sup35 in Saccharomyces cerevisiae.
588. Two prion variants of Sup35p have in-register parallel beta-sheet structures, independent of hydration.
589. Distinct spatial activation of intrinsic and extrinsic apoptosis pathways in natural scrapie: association with prion-related lesions.
590. Molecular pathology of human prion diseases.
591. Anti-PrP Mab 6D11 suppresses PrP(Sc) replication in prion infected myeloid precursor line FDC-P1/22L and in the lymphoreticular system in vivo.
592. Ultramicroscopy reveals axonal transport impairments in cortical motor neurons at prion disease.
593. EPR of Cu2+ prion protein constructs at 2 GHz using the g(perpendicular) region to characterize nitrogen ligation.
594. Synthetic lipid vesicles recruit native-like aggregates and affect the aggregation process of the prion Ure2p: insights on vesicle permeabilization and charge selectivity.
595. Early onset prion disease from octarepeat expansion correlates with copper binding properties.
596. Heterozygous inhibition in prion infection: the stone fence model.
597. Role of the lymphoreticular system in prion neuroinvasion from the oral and nasal mucosa.
598. Transport of the pathogenic prion protein through landfill materials.
599. [The information embedded into protein conformation: the mammalian prion protein is not the only one which "prionized"].
600. Proteasome inhibitors promote the sequestration of PrPSc into aggresomes within the cytosol of prion-infected CAD neuronal cells.
601. Fishing for prion protein function.
602. Paracrine diffusion of PrP(C) and propagation of prion infectivity by plasma membrane-derived microvesicles.
603. Rapid fracture healing in a patient with inherited prion disease.
604. Prion protein on astrocytes or in extracellular fluid impedes neurodegeneration induced by truncated prion protein.
605. Heterologous prion interactions are altered by mutations in the prion protein Rnq1p.
606. Thalamo-striatal diffusion reductions precede disease onset in prion mutation carriers.
607. Novel glutaredoxin activity of the yeast prion protein Ure2 reveals a native-like dimer within fibrils.
608. Folding kinetics of the human prion protein probed by temperature jump.
609. Doppel induces autophagic stress in prion protein-deficient Purkinje cells.
610. A landmark systems analysis of prion disease of the brain.
611. A systems approach to prion disease.
612. Heterologous stacking of prion protein peptides reveals structural details of fibrils and facilitates complete inhibition of fibril growth.
613. Anti-idiotypic antibodies: a new approach in prion research.
614. Prion protein glycosylation is not required for strain-specific neurotropism.
615. Ovine progressive pneumonia provirus levels are unaffected by the prion 171R allele in an Idaho sheep flock.
616. Abnormal brain iron homeostasis in human and animal prion disorders.
617. Cytoplasmic prion protein induces forebrain neurotoxicity.
618. Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial.
619. Clinical trials for prion disease: difficult challenges, but hope for the future.
620. Regulation of embryonic cell adhesion by the prion protein.
621. Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion protein.
622. New inhibitors of prion replication that target the amyloid precursor.
623. The number and transmission of [PSI] prion seeds (Propagons) in the yeast Saccharomyces cerevisiae.
624. New insights into prion biology from the novel [SWI+] system.
625. Accumulation and dissemination of prion protein in experimental sheep scrapie in the natural host.
626. Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers.
627. Prion proteostasis: Hsp104 meets its supporting cast.
628. Prion diseases and their biochemical mechanisms.
629. Characterization of cell-surface prion protein relative to its recombinant analogue: insights from molecular dynamics simulations of diglycosylated, membrane-bound human prion protein.
630. Mutants of the Paf1 complex alter phenotypic expression of the yeast prion [PSI+].
631. The cellular concentration of the yeast Ure2p prion protein affects its propagation as a prion.
632. Cells expressing anchorless prion protein are resistant to scrapie infection.
633. Surface charge of polyoxometalates modulates polymerization of the scrapie prion protein.
634. Detection of typical and atypical bovine spongiform encephalopathy and scrapie prion strains by prion protein motif-grafted antibodies.
635. Cryptic peptides of the kringle domains preferentially bind to disease-associated prion protein.
636. The yeast global transcriptional co-repressor protein Cyc8 can propagate as a prion.
637. Unswitched immunoglobulin M response prolongs mouse survival in prion disease.
638. Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins.
639. HECTD2 is associated with susceptibility to mouse and human prion disease.
640. Prion protein modulates cellular iron uptake: a novel function with implications for prion disease pathogenesis.
641. Inoculation of scrapie with the self-assembling RADA-peptide disrupts prion accumulation and extends hamster survival.
642. The comprehensive native interactome of a fully functional tagged prion protein.
643. Crystal structure of human prion protein bound to a therapeutic antibody.
644. A role of cellular prion protein in programming T-cell cytokine responses in disease.
645. Recent advances in prion chemotherapeutics.
646. Helix 3 is necessary and sufficient for prion protein's anti-Bax function.
647. Dynamic interactions of Sup35p and PrP prion protein domains modulate aggregate nucleation and seeding.
648. Rapid diagnosis of human prion disease using streptomycin with tonsil and brain tissues.
649. Autophagy induction by trehalose counteracts cellular prion infection.
650. The region approximately between amino acids 81 and 137 of proteinase K-resistant PrPSc is critical for the infectivity of the Chandler prion strain.
651. A prion of yeast metacaspase homolog (Mca1p) detected by a genetic screen.
652. Dynamics of a truncated prion protein, PrP(113-231), from (15)N NMR relaxation: order parameters calculated and slow conformational fluctuations localized to a distinct region.
653. Methionine sulfoxides on prion protein Helix-3 switch on the alpha-fold destabilization required for conversion.
654. Effects of mutations in yeast prion [PSI+] on amyloid toxicity manifested in Escherichia coli strain BL21.
655. Prion sequence polymorphisms and chronic wasting disease resistance in Illinois white-tailed deer (Odocoileus virginianus).
656. Associations between lamb survival and prion protein genotype: analysis of data for ten sheep breeds in Great Britain.
657. Free tyrosine and tyrosine-rich peptide-dependent superoxide generation catalyzed by a copper-binding, threonine-rich neurotoxic peptide derived from prion protein.
658. Reversible monomer-oligomer transition in human prion protein.
659. Antagonistic roles of the N-terminal domain of prion protein to doppel.
660. Cdk5 acts as a mediator of neuronal cell cycle re-entry triggered by amyloid-beta and prion peptides.
661. Prion protein misfolding and disease.
662. The strength of selection against the yeast prion [PSI+].
663. Prion disease diagnosis by proteomic profiling.
664. Doppel-induced cytotoxicity in human neuronal SH-SY5Y cells is antagonized by the prion protein.
665. A G-protein gamma subunit mimic is a general antagonist of prion propagation in Saccharomyces cerevisiae.
666. Identification of adjacent binding sites for the YY1 and E4BP4 transcription factors in the ovine PrP (Prion) gene promoter.
667. Prion variants and species barriers among Saccharomyces Ure2 proteins.
668. Constraining the loop, releasing prion infectivity.
669. The yeast Sup35NM domain propagates as a prion in mammalian cells.
670. Detection of pathologic prion protein in the olfactory bulb of natural and experimental bovine spongiform encephalopathy affected cattle in Great Britain.
671. Olfactory behavior and physiology are disrupted in prion protein knockout mice.
672. Insights into intragenic and extragenic effectors of prion propagation using chimeric prion proteins.
673. Prion interference with multiple prion isolates.
674. Anti-bovine prion protein RNA aptamer containing tandem GGA repeat interacts both with recombinant bovine prion protein and its beta isoform with high affinity.
675. Left handed beta helix models for mammalian prion fibrils.
676. Cellular prion protein null mice display normal AMPA receptor mediated long term depression.
677. The peculiar interaction between mammalian prion protein and RNA.
678. Prion infection: seeded fibrillization or more?
679. Alkaline hydrolysis of mouse-adapted scrapie for inactivation and disposal of prion-positive material.
680. Strain-specific viral properties of variant Creutzfeldt-Jakob disease (vCJD) are encoded by the agent and not by host prion protein.
681. Casein kinase II interacts with prion protein in vitro and forms complex with native prion protein in vivo.
682. Pathogenic prion protein is degraded by a manganese oxide mineral found in soils.
683. Human prion protein (PrP) 219K is converted to PrPSc but shows heterozygous inhibition in variant Creutzfeldt-Jakob disease infection.
684. The NatA acetyltransferase couples Sup35 prion complexes to the [PSI+] phenotype.
685. Bacterial colitis increases susceptibility to oral prion disease.
686. Prion switching in response to environmental stress.
687. ATM-mediated transcriptional elevation of prion in response to copper-induced oxidative stress.
688. The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes.
689. Biosynthesis of prion protein nucleocytoplasmic isoforms by alternative initiation of translation.
690. Detection of prion infectivity in fat tissues of scrapie-infected mice.
691. Urinary alpha1-antichymotrypsin: a biomarker of prion infection.
692. The type I Hsp40 Ydj1 utilizes a farnesyl moiety and zinc finger-like region to suppress prion toxicity.
693. Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities.
694. Transmission of atypical bovine prions to mice transgenic for human prion protein.
695. In vivo monitoring of the prion replication cycle reveals a critical role for Sis1 in delivering substrates to Hsp104.
696. Prion propagation in vitro: are we there yet?
697. Curing of yeast [URE3] prion by the Hsp40 cochaperone Ydj1p is mediated by Hsp70.
698. Variety of antiprion compounds discovered through an in silico screen based on cellular-form prion protein structure: Correlation between antiprion activity and binding affinity.
699. Histidine at codon 154 of the prion protein gene is a risk factor for Nor98 scrapie in goats.
700. Development of antibody fragments for immunotherapy of prion diseases.
701. Regulation of prion gene expression by transcription factors SP1 and metal transcription factor-1.
702. A miRNA signature of prion induced neurodegeneration.
703. Specific biarsenical labeling of cell surface proteins allows fluorescent- and biotin-tagging of amyloid precursor protein and prion proteins.
704. Prion strain targeting independent of strain-specific neuronal tropism.
705. Ligand binding promotes prion protein aggregation--role of the octapeptide repeats.
706. Wasting and neurologic signs in a white-tailed deer (Odocoileus virginianus) not associated with abnormal prion protein.
707. Ablation of prion protein immunoreactivity by heating in saturated calcium hydroxide.
708. A novel PRNP-P105S mutation associated with atypical prion disease and a rare PrPSc conformation.
709. Immune-directed gene therapeutic development for Alzheimer's, prion, and Parkinson's diseases.
710. Prion protein amyloid formation under native-like conditions involves refolding of the C-terminal alpha-helical domain.
711. Human prion disease with a G114V mutation and epidemiological studies in a Chinese family: a case series.
712. Reduction of prion infectivity in packed red blood cells.
713. Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease.
714. Review. The origin of the prion agent of kuru: molecular and biological strain typing.
715. Some recollections about kuru in a patient at Rabaul in 1978, and subsequent experiences with prion diseases.
716. The work of the Kuru Field Unit, Kuru Research Project of the Papua New Guinea Institute of Medical Research and MRC Prion unit.
717. Accelerated prion replication in, but prolonged survival times of, prion-infected CXCR3-/- mice.
718. Opposing roles of prion protein in oxidative stress- and ER stress-induced apoptotic signaling.
719. Curing of the [URE3] prion by Btn2p, a Batten disease-related protein.
720. Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
721. Absence of the cellular prion protein exacerbates and prolongs neuroinflammation in experimental autoimmune encephalomyelitis.
722. Reduced translocation of nascent prion protein during ER stress contributes to neurodegeneration.
723. Cell-free propagation of prion strains.
724. Evolution and differentiation of the prion protein gene (PRNP) among species.
725. The role of calorie restriction and SIRT1 in prion-mediated neurodegeneration.
726. The effects of prion protein proteolysis and disaggregation on the strain properties of hamster scrapie.
727. Modeling by assembly and molecular dynamics simulations of the low Cu2+ occupancy form of the mammalian prion protein octarepeat region: gaining insight into Cu2+-mediated beta-cleavage.
728. The polybasic N-terminal region of the prion protein controls the physical properties of both the cellular and fibrillar forms of PrP.
729. Docosahexaenoic and eicosapentaenoic acids increase prion formation in neuronal cells.
730. BSE case associated with prion protein gene mutation.
731. Both Met(109) and Met(112) are utilized for Cu(II) coordination by the amyloidogenic fragment of the human prion protein at physiological pH.
732. Genetic variability at seven codons of the prion protein gene in nine Pakistani sheep breeds.
733. Structural analysis of r(GGA)4 found in RNA aptamer for bovine prion protein.
734. Accelerated high fidelity prion amplification within and across prion species barriers.
735. A C-terminal protease-resistant prion fragment distinguishes ovine "CH1641-like" scrapie from bovine classical and L-Type BSE in ovine transgenic mice.
736. Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series.
737. Strain-specific sequences required for yeast [PSI+] prion propagation.
738. Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease.
739. Persistence of pathogenic prion protein during simulated wastewater treatment processes.
740. Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease.
741. Prion protein lacks robust cytoprotective activity in cultured cells.
742. Investigation of mcp1 as a quantitative trait gene for prion disease incubation time in mouse.
743. Accelerated prion disease pathogenesis in Toll-like receptor 4 signaling-mutant mice.
744. The cellular prion protein PrP(c) is involved in the proliferation of epithelial cells and in the distribution of junction-associated proteins.
745. A sequencing strategy for identifying variation throughout the prion gene of BSE-affected cattle.
746. Prion-impairing mutations in Hsp70 chaperone Ssa1: effects on ATPase and chaperone activities.
747. Prion infected meat-and-bone meal is still infectious after biodiesel production.
748. Prion gene (PRNP) haplotype variation in United States goat breeds (Open Access publication).
749. Acute cellular uptake of abnormal prion protein is cell type and scrapie-strain independent.
750. Identification of the copper(II) coordinating residues in the prion protein by metal-catalyzed oxidation mass spectrometry: evidence for multiple isomers at low copper(II) loadings.
751. Hydration effects on the HET-s prion and amyloid-beta fibrillous aggregates, studied with three-dimensional molecular theory of solvation.
752. Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin.
753. Novel dominant-negative prion protein mutants identified from a randomized library.
754. Scrapie-induced defects in learning and memory of transgenic mice expressing anchorless prion protein are associated with alterations in the gamma aminobutyric acid-ergic pathway.
755. Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains.
756. Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases.
757. Prion protein amino acid determinants of differential susceptibility and molecular feature of prion strains in mice and voles.
758. Effective gene therapy in a mouse model of prion diseases.
759. Prion diseases are efficiently transmitted by blood transfusion in sheep.
760. Delivery of single-chain antibodies (scFvs) directed against the 37/67 kDa laminin receptor into mice via recombinant adeno-associated viral vectors for prion disease gene therapy.
761. Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease.
762. UV-light exposed prion protein fails to form amyloid fibrils.
763. Prevalence of the prion protein gene E211K variant in U.S. cattle.
764. Binding of recombinant but not endogenous prion protein to DNA causes DNA internalization and expression in mammalian cells.
765. Altered neuron excitability and synaptic plasticity in the cerebellar granular layer of juvenile prion protein knock-out mice with impaired motor control.
766. Copper deficiency in the young bovine results in dramatic decreases in brain copper concentration but does not alter brain prion protein biology.
767. Cytosolic prion protein is the predominant anti-Bax prion protein form: exclusion of transmembrane and secreted prion protein forms in the anti-Bax function.
768. Endocytosis of prion protein is required for ERK1/2 signaling induced by stress-inducible protein 1.
769. A novel human disease with abnormal prion protein sensitive to protease.
770. Stress-protective signalling of prion protein is corrupted by scrapie prions.
771. Contribution of antibody and T cell-specific responses to the progression of 139A-scrapie in C57BL/6 mice immunized with prion protein peptides.
772. Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice.
773. Genes contributing to prion pathogenesis.
774. Nanomechanical properties of human prion protein amyloid as probed by force spectroscopy.
775. Misfolding of the prion protein: linking biophysical and biological approaches.
776. Cytoplasmic expression of mouse prion protein causes severe toxicity in Caenorhabditis elegans.
777. Prion agent diversity and species barrier.
778. GFP-tagged mutant prion protein forms intra-axonal aggregates in transgenic mice.
779. Retrotranslocation of prion proteins from the endoplasmic reticulum by preventing GPI signal transamidation.
780. Prions' travels--feces and transmission of prion diseases.
781. Prion protein attenuates excitotoxicity by inhibiting NMDA receptors.
782. All quiet on the neuronal front: NMDA receptor inhibition by prion protein.
783. Are cheetahs on the run from prion-like amyloidosis?
784. Chaperone-dependent amyloid assembly protects cells from prion toxicity.
785. Prion propagation in mice lacking central nervous system NF-kappaB signalling.
786. Effect of intraventricular infusion of anti-prion protein monoclonal antibodies on disease progression in prion-infected mice.
787. Cholesterol transporter ATP-binding cassette A1 (ABCA1) is elevated in prion disease and affects PrPC and PrPSc concentrations in cultured cells.
788. Prion protein immunohistochemistry in Creutzfeldt-Jakob disease.
789. [The role of the immune system in the pathogenesis of prion diseases].
790. The cellular prion protein is preferentially expressed by CD4+ CD25+ Foxp3+ regulatory T cells.
791. Prion protein complexed to N2a cellular RNAs through its N-terminal domain forms aggregates and is toxic to murine neuroblastoma cells.
792. All quiet on the neuronal front: NMDA receptor inhibition by prion protein.
793. Prion protein attenuates excitotoxicity by inhibiting NMDA receptors.
794. Molecular conformation and dynamics of the Y145Stop variant of human prion protein in amyloid fibrils.
795. Prominent pancreatic endocrinopathy and altered control of food intake disrupt energy homeostasis in prion diseases.
796. Host PrP glycosylation: a major factor determining the outcome of prion infection.
797. Cellular pathogenesis in prion diseases.
798. Association of the prion protein gene with individual tissue weights in Scottish Blackface sheep.
799. High titers of mucosal and systemic anti-PrP antibodies abrogate oral prion infection in mucosal-vaccinated mice.
800. Alterations in Ca2+-buffering in prion-null mice: association with reduced afterhyperpolarizations in CA1 hippocampal neurons.
801. The same primary structure of the prion protein yields two distinct self-propagating states.
802. Molecular chaperones and the assembly of the prion Ure2p in vitro.
803. Role of Erk1/2 activation in prion disease pathogenesis: absence of CCR1 leads to increased Erk1/2 activation and accelerated disease progression.
804. CRBL cells: establishment, characterization and susceptibility to prion infection.
805. Physiology of the prion protein.
806. The tubulovesicular structures - the ultrastructural hallmark for all prion diseases.
807. A prion disease of cervids: chronic wasting disease.
808. Antiprion prophylaxis by gene transfer of a soluble prion antagonist.
809. Prion protein insertional mutations increase aggregation propensity but not fiber stability.
810. The efficacy of tetracyclines in peripheral and intracerebral prion infection.
811. Newly identified prion linked to the chromatin-remodeling factor Swi1 in Saccharomyces cerevisiae.
812. Association of a bovine prion gene haplotype with atypical BSE.
813. Amyloid fibrils of the HET-s(218-289) prion form a beta solenoid with a triangular hydrophobic core.
814. Hsp110 chaperones regulate prion formation and propagation in S. cerevisiae by two discrete activities.
815. Manganese binding to the prion protein.
816. A regulatory role of the Rnq1 nonprion domain for prion propagation and polyglutamine aggregates.
817. Conformational change in hamster scrapie prion protein (PrP27-30) associated with proteinase K resistance and prion infectivity.
818. A real-time polymerase chain reaction assay to detect single nucleotide polymorphisms at codon 171 in the prion gene for the genotyping of scrapie susceptibility in sheep.
819. Prion protein in sheep urine.
820. Comprehensive transcriptional profiling of prion infection in mouse models reveals networks of responsive genes.
821. Semen from scrapie-infected rams does not transmit prion infection to transgenic mice.
822. Antiprion properties of prion protein-derived cell-penetrating peptides.
823. LRP1 controls biosynthetic and endocytic trafficking of neuronal prion protein.
824. Rodent models for prion diseases.
825. The evidence of associations between prion protein genotype and production, reproduction, and health traits in sheep.
826. NMR structure and CD titration with metal cations of human prion alpha2-helix-related peptides.
827. Amyloid of Rnq1p, the basis of the [PIN+] prion, has a parallel in-register beta-sheet structure.
828. Mechanisms of prion protein assembly into amyloid.
829. Expression of cellular isoform of prion protein on the surface of peripheral blood lymphocytes among women exposed to low doses of ionizing radiation.
830. Prion diseases: from protein to cell pathology.
831. Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain.
832. Conformational pH dependence of intermediate states during oligomerization of the human prion protein.
833. The elk PRNP codon 132 polymorphism controls cervid and scrapie prion propagation.
834. Evaluation of drugs for treatment of prion infections of the central nervous system.
835. Unfolded protein response transcription factor XBP-1 does not influence prion replication or pathogenesis.
836. Unraveling prion strains with cell biology and organic chemistry.
837. Prion disease induced alterations in gene expression in spleen and brain prior to clinical symptoms.
838. Pathologic prion protein infects cells by lipid-raft dependent macropinocytosis.
839. Comparative bioinformatics analysis of prion proteins isolated from reptile, rodent, ruminant, and human species.
840. Risk assessment of transmission of sporadic Creutzfeldt-Jakob disease in endodontic practice in absence of adequate prion inactivation.
841. Prion detection by an amyloid seeding assay.
842. A cell line infectible by prion strains from different species.
843. Human prion diseases: from antibody screening to a standardized fast immunodiagnosis using automation.
844. Prion strain discrimination in cell culture: the cell panel assay.
845. Cell models of prion infection.
846. Physiological role of the cellular prion protein.
847. A versatile prion replication assay in organotypic brain slices.
848. Prions and prion diseases: fundamentals and mechanistic details.
849. Deconvoluting the Cu2+ binding modes of full-length prion protein.
850. The role of Sse1 in the de novo formation and variant determination of the [PSI+] prion.
851. Squalestatin alters the intracellular trafficking of a neurotoxic prion peptide.
852. The prion protein is a combined zinc and copper binding protein: Zn2+ alters the distribution of Cu2+ coordination modes.
853. Diminishing apoptosis by deletion of Bax or overexpression of Bcl-2 does not protect against infectious prion toxicity in vivo.
854. Molecular architecture of human prion protein amyloid: a parallel, in-register beta-structure.
855. Prion propagation in a nerve conduit model containing segments devoid of axons.
856. Silencing of prion protein sensitizes breast adriamycin-resistant carcinoma cells to TRAIL-mediated cell death.
857. Prion protein regulates glutamate-dependent lactate transport of astrocytes.
858. Cell division modulates prion accumulation in cultured cells.
859. Induced prion protein controls immune-activated retroviruses in the mouse spleen.
860. Alternative assembly pathways of the amyloidogenic yeast prion determinant Sup35-NM.
861. Suppression of polyglutamine toxicity by the yeast Sup35 prion domain in Drosophila.
862. Classic scrapie in sheep with the ARR/ARR prion genotype in Germany and France.
863. Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis.
864. Highly promiscuous nature of prion polymerization.
865. The stability and aggregation of ovine prion protein associated with classical and atypical scrapie correlates with the ease of unwinding of helix-2.
866. Production of a soluble recombinant prion protein fused to blue fluorescent protein without refolding or detergents in Escherichia coli cells.
867. Assessment of prion inactivation by combined use of Bacillus-derived protease and SDS.
868. Prion protein/protein interactions: fusion with yeast Sup35p-NM modulates cytosolic PrP aggregation in mammalian cells.
869. Removal of the glycosylation of prion protein provokes apoptosis in SF126.
870. Hemin interactions and alterations of the subcellular localization of prion protein.
871. Prion strain- and species-dependent effects of antiprion molecules in primary neuronal cultures.
872. An emerging concept of prion infections as a form of transmissible cerebral amyloidosis.
873. Biological roles of prion domains.
874. Prion pathogenesis is independent of caspase-12.
875. Chaperone effects on prion and nonprion aggregates.
876. Interplays between covalent modifications in the endoplasmic reticulum increase conformational diversity in nascent prion protein.
877. Screening of DNA aptamer against mouse prion protein by competitive selection.
878. Role of Hsp104 in the propagation and inheritance of the [Het-s] prion.
879. Orally administered amyloidophilic compound is effective in prolonging the incubation periods of animals cerebrally infected with prion diseases in a prion strain-dependent manner.
880. Molecular biology of prion protein and its first homologous protein.
881. Influence of water, fat, and glycerol on the mechanism of thermal prion inactivation.
882. [Functions of prion protein PrPc].
883. Mutant prion protein D202N associated with familial prion disease is retained in the endoplasmic reticulum and forms 'curly' intracellular aggregates.
884. Efficacy of adjuvant chemotherapy according to Prion protein expression in patients with estrogen receptor-negative breast cancer.
885. Enzymatic detergent treatment protocol that reduces protease-resistant prion protein load and infectivity from surgical-steel monofilaments contaminated with a human-derived prion strain.
886. Intraepithelial and interstitial deposition of pathological prion protein in kidneys of scrapie-affected sheep.
887. Normal prion protein trafficking in cultured human erythroblasts.
888. Detection of the disease-associated isoform of the prion protein in formalin-fixed tissues by Western blot.
889. Nonpsychoactive cannabidiol prevents prion accumulation and protects neurons against prion toxicity.
890. In vitro and in vivo neurotoxicity of prion protein oligomers.
891. Crystallization and preliminary X-ray crystallographic analysis of a 40 kDa N-terminal fragment of the yeast prion-remodeling factor Hsp104.
892. The dominant-negative effect of the Q218K variant of the prion protein does not require protein X.
893. Mouse-adapted ovine scrapie prion strains are characterized by different conformers of PrPSc.
894. Polymorphisms of the prion gene promoter region that influence classical bovine spongiform encephalopathy susceptibility are not applicable to other transmissible spongiform encephalopathies in cattle.
895. Polylactide-coglycolide microspheres co-encapsulating recombinant tandem prion protein with CpG-oligonucleotide break self-tolerance to prion protein in wild-type mice and induce CD4 and CD8 T cell responses.
896. Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain.
897. Molecular dynamics simulations of two tandem octarepeats from the mammalian prion protein: fully Cu2+-bound and metal-free forms.
898. The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections.
899. Cyclodextrins inhibit replication of scrapie prion protein in cell culture.
900. Amyloid-specific fluorophores for the rapid, sensitive in situ detection of prion contamination on surgical instruments.
901. Assessment of prion inactivation by fenton reaction using protein misfolding cyclic amplification and bioassay.
902. Identification of single-nucleotide polymorphisms of the prion protein gene in sika deer (Cervus nippon laiouanus).
903. J-protein co-chaperone Sis1 required for generation of [RNQ+] seeds necessary for prion propagation.
904. Aggregation of cellular prion protein is initiated by proximity-induced dimerization.
905. A novel real-time ultrasonic method for prion protein detection using plasminogen as a capture molecule.
906. Knockdown of the bovine prion gene PRNP by RNA interference (RNAi) technology.
907. Mechanistic insights into the cure of prion disease by novel antiprion compounds.
908. Prion protein expression differences in microglia and astroglia influence scrapie-induced neurodegeneration in the retina and brain of transgenic mice.
909. Molecular profiling of ovine prion diseases by using thermolysin-resistant PrPSc and endogenous C2 PrP fragments.
910. Exposure to low dietary copper or low copper coupled with high dietary manganese for one year does not alter brain prion protein characteristics in the mature cow.
911. Cellular prion protein (PrPC) protects neuronal cells from the effect of huntingtin aggregation.
912. PrPc does not mediate internalization of PrPSc but is required at an early stage for de novo prion infection of Rov cells.
913. Experimental scrapie in 'plt' mice: an assessment of the role of dendritic-cell migration in the pathogenesis of prion diseases.
914. Oral transmissibility of prion disease is enhanced by binding to soil particles.
915. Hot spots in prion protein for pathogenic conversion.
916. A non-Q/N-rich prion domain of a foreign prion, [Het-s], can propagate as a prion in yeast.
917. Cell division is essential for elimination of the yeast [PSI+] prion by guanidine hydrochloride.
918. Generation of human scFvs antibodies recognizing a prion protein epitope expressed on the surface of human lymphoblastoid cells.
919. Characterization of prion protein (PrP)-derived peptides that discriminate full-length PrPSc from PrPC.
920. A structural overview of the vertebrate prion proteins.
921. Prion and nonprion amyloids: a comparison inspired by the yeast Sup35 protein.
922. Prion stability.
923. Prion-prion interactions.
924. A bovine prion acquires an epidemic bovine spongiform encephalopathy strain-like phenotype on interspecies transmission.
925. Phosphorothioate oligonucleotides reduce PrP levels and prion infectivity in cultured cells.
926. Alteration of NF-kappaB activity leads to mitochondrial apoptosis after infection with pathological prion protein.
927. Cellular prion protein regulates beta-secretase cleavage of the Alzheimer's amyloid precursor protein.
928. Contributions of neuronal prion protein on sleep recovery and stress response following sleep deprivation.
929. Modulation of proteinase K-resistant prion protein in cells and infectious brain homogenate by redox iron: implications for prion replication and disease pathogenesis.
930. Diversity in neuroanatomical distribution of abnormal prion protein in atypical scrapie.
931. Characterization of the properties and trafficking of an anchorless form of the prion protein.
932. Dendritic pathology in prion disease starts at the synaptic spine.
933. Levels of abnormal prion protein in deer and elk with chronic wasting disease.
934. Prion protein repeat expansion results in increased aggregation and reveals phenotypic variability.
935. Characterization of the genomic region containing the Shadow of Prion Protein (SPRN) gene in sheep.
936. Motor behavioral and neuropathological deficits in mice deficient for normal prion protein expression.
937. Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
938. Investigating the relationship between the prion protein locus and udder morphology traits and milk yield in Sardinian sheep.
939. Ser170 controls the conformational multiplicity of the loop 166-175 in prion proteins: implication for conversion and species barrier.
940. Species-specific differences in the intermediate states of human and Syrian hamster prion protein detected by high pressure NMR spectroscopy.
941. Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice.
942. Ure2p function is enhanced by its prion domain in Saccharomyces cerevisiae.
943. Cellular prion protein interaction with vitronectin supports axonal growth and is compensated by integrins.
944. Normal cellular prion protein is a ligand of selectins: binding requires Le(X) but is inhibited by sLe(X).
945. Prion recognition elements govern nucleation, strain specificity and species barriers.
946. Prion protein genes in caribou from Alaska.
947. Bcl-2 overexpression delays caspase-3 activation and rescues cerebellar degeneration in prion-deficient mice that overexpress amino-terminally truncated prion.
948. Defective retrotranslocation causes loss of anti-Bax function in human familial prion protein mutants.
949. Cellular prion protein and caveolin-1 interaction in a neuronal cell line precedes Fyn/Erk 1/2 signal transduction.
950. [Sleep disorders in prion diseases].
951. Recent developments in prion disease research: diagnostic tools and in vitro cell culture models.
952. Molecular dynamics simulations on the oligomer-formation process of the GNNQQNY peptide from yeast prion protein Sup35.
953. Structural and hydration properties of the partially unfolded states of the prion protein.
954. Normal cellular prion protein protects against manganese-induced oxidative stress and apoptotic cell death.
955. Nanoengineered analytical immobilized metal affinity chromatography stationary phase by atom transfer radical polymerization: separation of synthetic prion peptides.
956. Scrapie infection of prion protein-deficient cell line upon ectopic expression of mutant prion proteins.
957. Development of an assay to determine single nucleotide polymorphisms in the prion gene for the genetic diagnosis of relative susceptibility to classical scrapie in sheep.
958. Human prion proteins with pathogenic mutations share common conformational changes resulting in enhanced binding to glycosaminoglycans.
959. The cellular prion protein (PrP(C)): its physiological function and role in disease.
960. Diversity in prion protein oligomerization pathways results from domain expansion as revealed by hydrogen/deuterium exchange and disulfide linkage.
961. Prion protein alleles showing a protective effect on the susceptibility of sheep to scrapie and bovine spongiform encephalopathy.
962. Prophylactic effect of dietary seaweed Fucoidan against enteral prion infection.
963. What history tells us VIII. The progressive construction of a mechanism for prion diseases.
964. Mapping of possible prion protein self-interaction domains using peptide arrays.
965. M1 and M3 muscarinic receptors control physiological processing of cellular prion by modulating ADAM17 phosphorylation and activity.
966. Inducible overexpression of wild-type prion protein in the muscles leads to a primary myopathy in transgenic mice.
967. Propagation of the [PIN+] prion by fragments of Rnq1 fused to GFP.
968. Prevention of prion propagation by dehydrocholesterol reductase inhibitors in cultured cells and a therapeutic trial in mice.
969. Cellular prion protein promotes proliferation and G1/S transition of human gastric cancer cells SGC7901 and AGS.
970. Prion disease in Sri Lanka.
971. Probing structural differences in prion protein isoforms by tyrosine nitration.
972. Yeast prions: evolution of the prion concept.
973. The genetic control of the formation and propagation of the [PSI+] prion of yeast.
974. The prion protein knockout mouse: a phenotype under challenge.
975. A short history of small s: a prion of the fungus Podospora anserina.
976. Prion-dependent lethality of sup45 mutants in Saccharomyces cerevisiae.
977. Nucleotide exchange factors for Hsp70s are required for [URE3] prion propagation in Saccharomyces cerevisiae.
978. The toxicity of the PrP106-126 prion peptide on cultured photoreceptors correlates with the prion protein distribution in the mammalian and human retina.
979. The role of the octarepeat region in neuroprotective function of the cellular prion protein.
980. CpG oligodeoxynucleotide-enhanced humoral immune response and production of antibodies to prion protein PrPSc in mice immunized with 139A scrapie-associated fibrils.
981. Discriminating between cellular and misfolded prion protein by using affinity to 9-aminoacridine compounds.
982. Progression of prion infectivity in asymptomatic cattle after oral bovine spongiform encephalopathy challenge.
983. Polymorphisms of the prion protein gene coding region in born-after-the-reinforced-ban (BARB) bovine spongiform encephalopathy cattle in Great Britain.
984. Ophthalmic surgery in prion diseases.
985. Similar biochemical signatures and prion protein genotypes in atypical scrapie and Nor98 cases, France and Norway.
986. Reversibility of prion-induced neurodegeneration.
987. Accumulation of prion protein in the brain that is not associated with transmissible disease.
988. Conversion of the BASE prion strain into the BSE strain: the origin of BSE?
989. Fragment length influences affinity for Cu2+ and Ni2+ binding to His96 or His111 of the prion protein and spectroscopic evidence for a multiple histidine binding only at low pH.
990. Peripheral circulation of the prion infectious agent in transgenic mice expressing the ovine prion protein gene in neurons only.
991. Bovine spongiform encephalopathy agent in a prion protein (PrP)ARR/ARR genotype sheep after peripheral challenge: complete immunohistochemical analysis of disease-associated PrP and transmission studies to ovine-transgenic mice.
992. Perturbation of endoplasmic reticulum homeostasis facilitates prion replication.
993. Diffusion of protease into meat & bone meal for solubility improvement and potential inactivation of the BSE prion.
994. Abnormal prion protein in the pituitary in sporadic and variant Creutzfeldt-Jakob disease.
995. Comparison of CR36, a new heparan mimetic, and pentosan polysulfate in the treatment of prion diseases.
996. Hsp40 interacts directly with the native state of the yeast prion protein Ure2 and inhibits formation of amyloid-like fibrils.
997. Prion infection of muscle cells in vitro.
998. Resistance to chronic wasting disease in transgenic mice expressing a naturally occurring allelic variant of deer prion protein.
999. Familial prion disease in a Hungarian family with a novel 144-base pair insertion in the prion protein gene.
1000. The nasal cavity is a route for prion infection in hamsters.