title: Biology and Genetics of PrP Prion Strains.
abstract: Prion diseases are a group of fatal neurodegenerative disorders caused by the misfolding of the cellular prion protein (PrPC) into a pathogenic conformation (PrPSc). PrPScis capable of folding into multiple self-replicating prion strains that produce phenotypically distinct neurological disorders. Evidence suggests that the structural heterogeneity of PrPScis the molecular basis of strain-specific prion properties. The self-templating of PrPSctypically ensures that prion strains breed true upon passage. However, prion strains also have the capacity to conformationally transform to maximize their rate of replication in a given environment. Here, we provide an overview of the prion-strain phenomenon and describe the role of strain adaptation in drug resistance. We also describe recent evidence that shows the presence of distinct conformational strains in other neurodegenerative disorders.
authors.affiliation: Department of Biology, University of Rochester, Rochester, New York 14627.
authors.forename: Sina
authors.initials: S
authors.lastname: Ghaemmaghami
mesh_terms: D000818:Animals; D004351:Drug Resistance; D006801:Humans; D017096:Prion Diseases / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D000072002:Prion Proteins / Q000235:genetics* / Q000378:metabolism*; D011487:Protein Conformation; D017510:Protein Folding
chemical_list: D000072002:Prion Proteins