title: Mouse models for studying the formation and propagation of prions.
abstract: Prions are self-propagating protein conformers that cause a variety of neurodegenerative disorders in humans and animals. Mouse models have played key roles in deciphering the biology of prions and in assessing candidate therapeutics. The development of transgenic mice that form prions spontaneously in the brain has advanced our understanding of sporadic and genetic prion diseases. Furthermore, the realization that many proteins can become prions has necessitated the development of mouse models for assessing the potential transmissibility of common neurodegenerative diseases. As the universe of prion diseases continues to expand, mouse models will remain crucial for interrogating these devastating illnesses.
authors.affiliation: From the Institute for Neurodegenerative Diseases and the Department of Neurology, University of California, San Francisco, California 94143.
authors.forename: Joel C
authors.initials: JC
authors.lastname: Watts
authors.affiliation: From the Institute for Neurodegenerative Diseases and the Department of Neurology, University of California, San Francisco, California 94143 stanley@ind.ucsf.edu.
authors.forename: Stanley B
authors.initials: SB
authors.lastname: Prusiner
mesh_terms: D000544:Alzheimer Disease / Q000209:etiology; D016229:Amyloid beta-Peptides / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D009154:Mutation; D010300:Parkinson Disease / Q000209:etiology; D017096:Prion Diseases / Q000209:etiology* / Q000378:metabolism / Q000628:therapy; D011328:Prions / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D051844:alpha-Synuclein / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D016875:tau Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism
chemical_list: D016229:Amyloid beta-Peptides; D011328:Prions; D051844:alpha-Synuclein; D016875:tau Proteins
keywords: Animal Model; Neurodegenerative Disease; Pathology; Prion; Protein Aggregation