Prion Diseases: From Protein to Cell Pathology
نویسندگان
چکیده
منابع مشابه
Cellular Prion Protein: From Physiology to Pathology
The human cellular prion protein (PrP(C)) is a glycosylphosphatidylinositol (GPI) anchored membrane glycoprotein with two N-glycosylation sites at residues 181 and 197. This protein migrates in several bands by Western blot analysis (WB). Interestingly, PNGase F treatment of human brain homogenates prior to the WB, which is known to remove the N-glycosylations, unexpectedly gives rise to two do...
متن کاملVisual pathology in animal prion diseases.
Prion diseases, also known as the transmissible spongiform encephalopathies (TSEs), are a group of slowly developing neurodegenerations occurring in human and animals. Prion diseases can be transferred between animals, humans, from humans to animals, and from animals to humans. As a result, the central nervous system is attacked, resulting in microglia activation, astrocytosis, prion plaque dep...
متن کاملThe molecular pathology of prion diseases.
15 Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal cellular prion protein (PrP) into a pathogenic likeness of itself (PrP). Formation of neurotoxic PrP and/or loss of the normal function o...
متن کاملMolecular Pathology of Human Prion Diseases
Prion diseases are fatal neurodegenerative conditions in humans and animals. In this review, we summarize the molecular background of phenotypic variability, relation of prion protein (PrP) to other proteins associated with neurodegenerative diseases, and pathogenesis of neuronal vulnerability. PrP exists in different forms that may be present in both diseased and non-diseased brain, however, a...
متن کاملPotential approaches for heterologous prion protein treatment of prion diseases
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: The American Journal of Pathology
سال: 2008
ISSN: 0002-9440
DOI: 10.2353/ajpath.2008.070442