نتایج جستجو برای: prion protein

تعداد نتایج: 1238350  

Journal: :Genetics 2012
Susan W Liebman Yury O Chernoff

The concept of a prion as an infectious self-propagating protein isoform was initially proposed to explain certain mammalian diseases. It is now clear that yeast also has heritable elements transmitted via protein. Indeed, the "protein only" model of prion transmission was first proven using a yeast prion. Typically, known prions are ordered cross-β aggregates (amyloids). Recently, there has be...

Journal: :Journal of virology 2000
K Doh-Ura T Iwaki B Caughey

We report that lysosomotropic agents and cysteine protease inhibitors inhibited protease-resistant prion protein accumulation in scrapie-infected neuroblastoma cells. The inhibition occurred without either apparent effects on normal prion protein biosynthesis or turnover or direct interactions with prion protein molecules. The findings introduce two new classes of inhibitors of the formation of...

Journal: :Archives of neurology 2007
Gianluigi Zanusso Alberto Polo Alessia Farinazzo Romolo Nonno Franco Cardone Michele Di Bari Sergio Ferrari Serena Principe Matteo Gelati Elisa Fasoli Michele Fiorini Frances Prelli Blas Frangione Giuseppe Tridente Marina Bentivoglio Alessandra Giorgi Maria Eugenia Schininà Bruno Maras Umberto Agrimi Nicola Rizzuto Maurizio Pocchiari Salvatore Monaco

OBJECTIVE To describe a novel molecular and pathological phenotype of Creutzfeldt-Jakob disease. Patient A 69-year-old woman with behavioral and personality changes followed by rapidly evolving dementia. RESULTS Postmortem examination of the brain showed intracellular prion protein deposition and axonal swellings filled with amyloid fibrils. Biochemical analysis of the pathological prion prot...

Journal: :Biochemistry 2002
P K Nandi E Leclerc D Marc

The unfolding of cellular prion protein and its refolding to the scrapie isoform are related to prion diseases. Studies in the literature have shown that structures of proteins, either acidic or basic, are stabilized against denaturation by certain neutral salts, for example, sulfate and fluoride. Contrary to these observations, the full-length recombinant prion protein (amino acid residues 23-...

2014
Marcelo A. Barria Aru Balachandran Masanori Morita Tetsuyuki Kitamoto Rona Barron Jean Manson Richard Knight James W. Ironside Mark W. Head

The risks posed to human health by individual animal prion diseases cannot be determined a priori and are difficult to address empirically. The fundamental event in prion disease pathogenesis is thought to be the seeded conversion of normal prion protein to its pathologic isoform. We used a rapid molecular conversion assay (protein misfolding cyclic amplification) to test whether brain homogena...

2013
Qian Ma Ji-Ying Hu Jie Chen Yi Liang

Prion diseases and prion-like protein misfolding diseases are related to the accumulation of abnormal aggregates of the normal host proteins including prion proteins and Tau protein. These proteins possess self-templating and transmissible characteristics. The crowded physiological environments where the aggregation of these amyloidogenic proteins takes place can be imitated in vitro by the add...

Journal: :Current Molecular Medicine 2009

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