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48 Newest Publications about the topic creutzfeldt-jakob disease


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Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases [Neuroscience]

03-Sep-2013 | Walker S. Jackson; Andrew W. Borkowski; Nicki E. Watson; Oliver D. King; Henryk Faas; Alan Jasanoff; Susan Lindquist, Proceedings of the National Academy of Sciences current issue, 2013

In man, mutations in different regions of the prion protein (PrP) are associated with infectious neurodegenerative diseases that have remarkably different clinical signs and neuropathological lesions. To explore the roots of this phenomenon, we created a knock-in mouse model carrying the mutation ...


PP2 and piceatannol inhibit PrP106–126-induced iNOS activation mediated by CD36 in BV2 microglia

01-Sep-2013 | Siming Zhang; Lifeng Yang; Mohammed Kouadir; Rongrong Tan; Yun Lu; Jiaxin Chang; Binrui Xu; Xiaomin Yin; Xiangmei Zh ..., Acta Biochimica et Biophysica Sinica, 2013

Prion diseases are a group of transmissible fatal neurodegenerative disorders of humans and animals, including bovine spongiform encephalopathy, scrapie, and Creutzfeldt–Jakob disease. Microglia, the resident macrophages of the central nervous system, are exquisitely sensitive to pathological ...


Molecular dynamics studies on 3D structures of the hydrophobic region PrP(109-136)

01-Jun-2013 | Jiapu Zhang; Yuanli Zhang, Acta Biochimica et Biophysica Sinica, 2013

Prion diseases, traditionally referred to as transmissible spongiform encephalopathies, are invariably fatal and highly infectious neurodegenerative diseases that affect a wide variety of mammalian species, manifesting as scrapie in sheep, bovine spongiform encephalopathy (or ‘mad-cow’ disease) ...


Unique drug screening approach for prion diseases identifies tacrolimus and astemizole as antiprion agents [Neuroscience]

23-Apr-2013 | Yervand Eduard Karapetyan; Gian Franco Sferrazza; Minghai Zhou; Gregory Ottenberg; Timothy Spicer; Peter Chase; Moha ..., Proceedings of the National Academy of Sciences current issue, 2013

Prion diseases such as Creutzfeldt–Jakob disease (CJD) are incurable and rapidly fatal neurodegenerative diseases. Because prion protein (PrP) is necessary for prion replication but dispensable for the host, we developed the PrP–FRET-enabled high throughput assay (PrP–FEHTA) to screen for ...


Thermodynamic analysis of structural transitions during GNNQQNY aggregation

14-Feb-2013 | Kenneth L. Osborne, Michael Bachmann, Birgit Strodel, Proteins: Structure, Function, and Bioinformatics, 2013

Abstract Amyloid protein aggregation characterizes many neurodegenerative disorders, including Alzheimer's, Parkinson's, and Creutzfeldt‐Jakob disease. Evidence suggests that amyloid aggregates may share similar aggregation pathways, implying simulation of full‐length amyloid proteins is not ...


Substitutions at residue 211 in the prion protein drive a switch between CJD and GSS syndrome, a new mechanism governing inherited neurodegenerative disorders

15-Dec-2012 | Katell Peoc'h; Etienne Levavasseur; Emilien Delmont; Alfonso De Simone; Isabelle Laffont-Proust; Nicolas Privat; Yas ..., Human Molecular Genetics, 2012

Human prion diseases are a heterogeneous group of fatal neurodegenerative disorders, characterized by the deposition of the partially protease-resistant prion protein (PrPres), astrocytosis, neuronal loss and spongiform change in the brain. Among inherited forms that represent 15% of patients, ...


sCJD subtype‐specific alterations of the brain proteome: impact on Rab3a recycling

16-Oct-2012 | Joanna Gawinecka, Franco Cardone, Abdul R. Asif, Angela De Pascalis, Wiebke M. Wemheuer, Walter J. Schulz‐Schaeffer, ..., PROTEOMICS, 2012

Sporadic Creutzfeldt‐Jakob disease (sCJD) is characterized by wide clinical and pathological variability, which is mainly influenced by the conformation of the misfolded prion protein, and by the methionine and valine polymorphism at codon 129 of the PRNP gene. This heterogeneity likely implies ...


Lack of association between 14-3-3 beta gene (YWHAB) polymorphisms and sporadic Creutzfeldt-Jakob disease (CJD)

10-Oct-2012 | Byung-Hoon Jeong, Hyoung-Tae Jin, Eun-Kyoung Choi, Richard I. Carp, Yong-Sun Kim, Molecular Biology Reports, 2012

14-3-3 proteins are highly abundant in brain tissue. The presence of 14-3-3 at elevated levels in the cerebrospinal fluid has been considered as a biomarker for sporadic Creutzfeldt-Jakob disease (CJD). Recent studies showed that 14-3-3 beta protein interacts with the N-terminal amino acids 1–38 ...


Overexpression of the Hspa13 (Stch) gene reduces prion disease incubation time in mice [Genetics]

21-Aug-2012 | Julia Grizenkova; Shaheen Akhtar; Holger Hummerich; Andrew Tomlinson; Emmanuel A. Asante; Adam Wenborn; Jérémie Fize ..., Proceedings of the National Academy of Sciences current issue, 2012

Prion diseases are fatal neurodegenerative disorders that include bovine spongiform encephalopathy (BSE) and scrapie in animals and Creutzfeldt-Jakob disease (CJD) in humans. They are characterized by long incubation periods, variation in which is determined by many factors including genetic ...


Small Protease Sensitive Oligomers of PrPSc in Distinct Human Prions Determine Conversion Rate of PrPC

02-Aug-2012 | Chae Kim et al., PLoS Pathogens, 2012

by Chae Kim, Tracy Haldiman, Krystyna Surewicz, Yvonne Cohen, Wei Chen, Janis Blevins, Man-Sun Sy, Mark Cohen, Qingzhong Kong, Glenn C. Telling, Witold K. Surewicz, Jiri G. Safar The mammalian prions replicate by converting cellular prion protein (PrPC) into pathogenic conformational ...


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