Associated Papers
Substitution of histidine 95 by tyrosine in the prion protein causes spontaneous neurodegeneration in transgenic mice.
Torres JM et al.
Plos Pathogens, 2025
Slow Misfolding of a Molten Globule form of a Mutant Prion Protein Variant into a β-rich Dimer.
Pal S et al.
Journal of Molecular Biology, 2024
Accumulation Area of a Japanese PRNP P102L Variant Associated With Gerstmann-Sträussler-Scheinker Disease: The Ariake PRNP P102L Variant.
Suzuyama K et al.
Journal of Clinical Neurology (seoul, Korea), 2024
Retraction Notice to: The Pathogenic A116V Mutation Enhances Ion-Selective Channel Formation by Prion Protein in Membranes.
Sabareesan AT et al.
Biophysical Journal, 2021
Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease.
Ximelis T et al.
Alzheimer's Research & Therapy, 2021
N-Terminal Regions of Prion Protein: Functions and Roles in Prion Diseases.
Hara H et al.
International Journal of Molecular Sciences, 2020
Intrinsic toxicity of the cellular prion protein is regulated by its conserved central region.
Roseman GP et al.
Faseb Journal : Official Publication of the Federation of American Societies for Experimental Biology, 2020
Novel Variations in Native Ethiopian Goat breeds PRNP Gene and Their Potential Effect on Prion Protein Stability.
Teferedegn EY et al.
Scientific Reports, 2020
Mutations in Prion Protein Gene: Pathogenic Mechanisms in C-Terminal vs. N-Terminal Domain, a Review.
Bernardi L et al.
International Journal of Molecular Sciences, 2019
Structural Determinants of the Prion Protein N-Terminus and Its Adducts with Copper Ions.
Sánchez-López C et al.
International Journal of Molecular Sciences, 2018
Structural mechanisms of oligomer and amyloid fibril formation by the prion protein.
Sengupta I et al.
Chemical Communications (cambridge, England), 2018
Loss of STI1-mediated neuronal survival and differentiation in disease-associated mutations of prion protein.
Landemberger MC et al.
Journal of Neurochemistry, 2018
Lysosomal Quality Control in Prion Diseases.
Majumder P et al.
Molecular Neurobiology, 2018
A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.
Mercer RCC et al.
Plos Pathogens, 2018
Experimental Models of Inherited PrP Prion Diseases.
Watts JC et al.
Cold Spring Harbor Perspectives in Medicine, 2017
Cellular prion protein is present in mitochondria of healthy mice.
Faris R et al.
Scientific Reports, 2017
Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.
Moreno JA et al.
Methods in Molecular Biology (clifton, N.j.), 2017
Transmissibility of Gerstmann-Sträussler-Scheinker syndrome in rodent models: New insights into the molecular underpinnings of prion infectivity.
Nonno R et al.
Prion, 2016
Pathogenic Mutations within the Disordered Palindromic Region of the Prion Protein Induce Structure Therein and Accelerate the Formation of Misfolded Oligomers.
Sabareesan AT et al.
Journal of Molecular Biology, 2016
The Pathogenic A116V Mutation Enhances Ion-Selective Channel Formation by Prion Protein in Membranes.
Sabareesan AT et al.
Biophysical Journal, 2016
Interplay of endoplasmic reticulum stress and autophagy in neurodegenerative disorders.
Cai Y et al.
Autophagy, 2016
Pathogenic mutations within the hydrophobic domain of the prion protein lead to the formation of protease-sensitive prion species with increased lethality.
Coleman BM et al.
Journal of Virology, 2014
The prion protein modulates A-type K+ currents mediated by Kv4.2 complexes through dipeptidyl aminopeptidase-like protein 6.
Mercer RC et al.
The Journal of Biological Chemistry, 2013
Role of Prion Disease-Linked Mutations in the Intrinsically Disordered N-Terminal Domain of the Prion Protein.
Cong X et al.
Journal of Chemical Theory and Computation, 2013
Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases.
Reiniger L et al.
Acta Neuropathologica Communications, 2013
Early Delivery of Misfolded PrP from ER to Lysosomes by Autophagy.
Cortes CJ et al.
International Journal of Cell Biology, 2013
Disease-associated mutations in the prion protein impair laminin-induced process outgrowth and survival.
Machado CF et al.
The Journal of Biological Chemistry, 2012
Rapamycin delays disease onset and prevents PrP plaque deposition in a mouse model of Gerstmann-Sträussler-Scheinker disease.
Cortes CJ et al.
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 2012
Prion protein facilitates uptake of zinc into neuronal cells.
Watt NT et al.
Nature Communications, 2012
An N-terminal polybasic domain and cell surface localization are required for mutant prion protein toxicity.
Solomon IH et al.
The Journal of Biological Chemistry, 2011
The prion diseases.
Brown K et al.
Journal of Geriatric Psychiatry and Neurology, 2010
A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.
Yang W et al.
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 2009
Coexpression of wild-type and mutant prion proteins alters their cellular localization and partitioning into detergent-resistant membranes.
Schiff E et al.
Traffic (copenhagen, Denmark), 2008
Cellular prion protein regulates beta-secretase cleavage of the Alzheimer's amyloid precursor protein.
Parkin ET et al.
Proceedings of the National Academy of Sciences of the United States of America, 2007
Reactive oxygen species (ROS)-mediated beta-cleavage of the prion protein in the mechanism of the cellular response to oxidative stress.
Watt NT et al.
Biochemical Society Transactions, 2005
Reactive oxygen species-mediated beta-cleavage of the prion protein in the cellular response to oxidative stress.
Watt NT et al.
The Journal of Biological Chemistry, 2005
Mutational analysis of topological determinants in prion protein (PrP) and measurement of transmembrane and cytosolic PrP during prion infection.
Stewart RS et al.
The Journal of Biological Chemistry, 2003
Most pathogenic mutations do not alter the membrane topology of the prion protein.
Stewart RS et al.
The Journal of Biological Chemistry, 2001
Ion channels induced by the prion protein: mediators of neurotoxicity.
Solomon IH et al.
Prion, 0