PATHOLOGIC CONFORMATIONS OF PRION PROTEINS
نویسندگان
چکیده
منابع مشابه
Deadly Conformations—Protein Misfolding in Prion Disease
and progressive dementia, occurring after age 40, associated , as in kuru, with plaques in the brain of affected * Department of Genetics and Howard Hughes Medical Institute individuals (Gerstmann et al., 1936). Multiple affected family members were observed, in a pattern indicating Yale University School of Medicine New Haven, Connecticut 06510 autosomal-dominant inheritance. Similar genetic t...
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The main-chain conformations of 237 384 amino acids in 1042 protein subunits from the PDB were analyzed with Ramachandran plots. The populated areas of the empirical Ramachandran plot differed markedly from the classical plot in all regions. All amino acids in alpha-helices are found within a very narrow range of phi, psi angles. As many as 40% of all amino acids are found in this most populate...
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Prion diseases such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, and scrapie in sheep are fatal neurodegenerative diseases for which there is no effective treatment. The pathology of these diseases involves the conversion of a protease sensitive form of the cellular prion protein (PrPC) into a protease resistant infectious form (PrPsc or PrPres). Both in v...
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ژورنال
عنوان ژورنال: Annual Review of Biochemistry
سال: 1998
ISSN: 0066-4154,1545-4509
DOI: 10.1146/annurev.biochem.67.1.793