Aggregated, Wild-Type Prion Protein Causes Neurological Dysfunction and Synaptic Abnormalities

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منابع مشابه

Neurobiology of Disease Aggregated, Wild-Type Prion Protein Causes Neurological Dysfunction and Synaptic Abnormalities

Roberto Chiesa,1 Pedro Piccardo,2,3 Emiliano Biasini,1,4 Bernardino Ghetti,2 and David A. Harris4 1Dulbecco Telethon Institute and Department of Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri, 20156, Milan, Italy, 2Indiana Alzheimer Disease Center, Indiana University School of Medicine, Indianapolis, Indiana 46202, 3Center for Biologics Evaluation and Research, Food and Drug Admi...

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Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities.

The neurotoxic forms of the prion protein (PrP) that cause neurodegeneration in prion diseases remain to be conclusively identified. Considerable evidence points to the importance of noninfectious oligomers of PrP in the pathogenic process. In this study, we describe lines of Tg(WT) transgenic mice that over-express wild-type PrP by either approximately 5-fold or approximately 10-fold (dependin...

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The structural stability of wild-type horse prion protein.

Prion diseases (e.g. Creutzfeldt-Jakob disease (CJD), variant CJD (vCJD), Gerstmann-Straussler-Scheinker syndrome (GSS), Fatal Familial Insomnia (FFI) and Kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE or 'mad-cow' disease) and chronic wasting disease (CWD) in cattles) are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. Ho...

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Expression of the Prion Protein Family Member Shadoo Causes Drug Hypersensitivity That Is Diminished by the Coexpression of the Wild Type Prion Protein.

The prion protein (PrP) seems to exert both neuroprotective and neurotoxic activities. The toxic activities are associated with the C-terminal globular parts in the absence of the flexible N terminus, specifically the hydrophobic domain (HD) or the central region (CR). The wild type prion protein (PrP-WT), having an intact flexible part, exhibits neuroprotective qualities by virtue of diminishi...

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P176: Neurological Diseases: Causes, Symptoms and Treatments

The nervous system is an extremely complex communication system that can send and receive large amounts of information simultaneously. The nervous system has two distinct parts: the central nervous system (the brain and the spinal cord) and the peripheral nervous system (the nerves located outside the brain and spinal cord). The main unit of the nervous system is neural cells (neurons). The rou...

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ژورنال

عنوان ژورنال: Journal of Neuroscience

سال: 2008

ISSN: 0270-6474,1529-2401

DOI: 10.1523/jneurosci.3109-08.2008