Immunomodulation for prion and prion-related diseases

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Prion and Prion-like Diseases in Humans

Introduction. Genetic prion diseases, such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), Fatal Familial Insomnia (FFI), are associated with the mutations of prion (PRNP) gene. More than 30 pathogenic PRNP mutations were identified. Since Alzheimer disease (AD) and prion diseases have many similarities in the neuropathological and physiological symptoms, we te...

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Stanley Prusiner introduced the term prion (proteinaceous infectious particle) and proposed that the infectious agent in prion diseases is composed mainly or entirely of an abnormal conformation of an otherwise normal host-encoded glycoprotein called the prion protein (PrP)1. This hypothesis was initially greeted with great skepticism in the scientific community but then later was widely acknow...

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Prion protein NMR structure and species barrier for prion diseases.

The structural basis of species specificity of transmissible spongiform encephalopathies, such as bovine spongiform encephalopathy or "mad cow disease" and Creutzfeldt-Jakob disease in humans, has been investigated using the refined NMR structure of the C-terminal domain of the mouse prion protein with residues 121-231. A database search for mammalian prion proteins yielded 23 different sequenc...

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Copper and prion diseases.

Transmissible spongiform encephalopathies are diseases of animals and humans that are also termed prion diseases. These diseases are linked together because a normal brain glycoprotein termed the prion protein is converted to a readily detectable protease-resistant isoform. There is now strong evidence to suggest that apart from this difference in resistance a major difference between the isofo...

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Prion diseases, or transmissible spongiform encephalopathies (TSEs) are progressive, fatal neurodegenerative diseases with no effective treatment. The pathology of these diseases involves the conversion of a protease sensitive form of the cellular prion protein (PrP(C)) into a protease resistant infectious form (PrP(res)). The efficiency of this conversion is predicated upon a number of factors...

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ژورنال

عنوان ژورنال: Expert Review of Vaccines

سال: 2010

ISSN: 1476-0584,1744-8395

DOI: 10.1586/erv.10.131