نتایج جستجو برای: human prion protein

تعداد نتایج: 2481093  

2015
Peter Rudge Zane Jaunmuktane Peter Adlard Nina Bjurstrom Diana Caine Jessica Lowe Penny Norsworthy Holger Hummerich Ron Druyeh Jonathan D. F. Wadsworth Sebastian Brandner Harpreet Hyare Simon Mead John Collinge

Patients with iatrogenic Creutzfeldt-Jakob disease due to administration of cadaver-sourced growth hormone during childhood are still being seen in the UK 30 years after cessation of this treatment. Of the 77 patients who have developed iatrogenic CreutzfeldtJakob disease, 56 have been genotyped. There has been a marked change in genotype profile at polymorphic codon 129 of the prion protein ge...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2003
Carlos J Bosques Barbara Imperiali

It is now accepted that the structural transition from cellular prion protein (PrPC) to proteinase K-resistant prion protein scrapie (PrPSc) is the major event leading to transmissible spongiform encephalopathies. Although the mechanism of this transition remains elusive, glycosylation has been proposed to impede the PrPC to PrPSc conversion. To address the role of glycosylation, we have prepar...

Journal: :Journal of Neuropathology and Experimental Neurology 2005

Journal: :Journal of Biological Chemistry 2010

2011
Eva Dvorakova Marek Prouza Olga Janouskova Martin Panigaj Karel Holada

Transmissive spongiform encephalopathies (TSE) are neurodegenerative diseases characterized by depositions of abnormally folded prion protein (PrP(TSE)) in brain. PrP(TSE) is at present the only specific biochemical marker of human and animal TSE. As deposits of PrP(TSE) remain in the body for long periods, there is substantial chance of them being nonenzymatically modified by glycation. The de...

Journal: :Journal of neurology, neurosurgery, and psychiatry 2013
Genevieve M J A Klug Handan Wand Marion Simpson Alison Boyd Matthew Law Colin L Masters Radoslav Matěj Rachel Howley Michael Farrell Maren Breithaupt Inga Zerr Cornelia van Duijn Carla Ibrahim-Verbaas Jan Mackenzie Robert G Will Jean-Philippe Brandel Annick Alperovitch Herbert Budka Gabor G Kovacs Gerard H Jansen Michael Coulthard Steven J Collins

BACKGROUND Prospective national screening and surveillance programmes serve a range of public health functions. Objectively determining their adequacy and impact on disease may be problematic for rare disorders. We undertook to assess whether objective measures of disease surveillance intensity could be developed for the rare disorder sporadic Creutzfeldt-Jakob disease (CJD) and whether such me...

2013
Richard Rubenstein Binggong Chang

Human prion diseases are fatal neurodegenerative disorders associated with an accumulation of PrP(Sc) in the central nervous system (CNS). Of the human prion diseases, sporadic Creutzfeldt-Jakob disease (sCJD), which has no known origin, is the most common form while variant CJD (vCJD) is an acquired human prion disease reported to differ from other human prion diseases in its neurological, neu...

Journal: :Electrophoresis 2012
Pavlina Sobrova Marketa Ryvolova Vojtech Adam Rene Kizek

Transmissible spongiform encephalopathies are a group of fatal neurodegenerative diseases with long incubation time. This group includes Creutzfeld-Jakob disease, kuru, scrapie, chronic wasting disease, and bovine spongiform encephalopathy. Sensitive and specific detection of abnormal prion protein as "a source agent" of the above-mentioned diseases in blood could provide a diagnostic test or a...

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