title: Prion proteins meet protein quality control.
abstract: Recent work on transmissible spongiform encephalopathies (TSEs) suggests a role for protein quality-control mechanisms in both prion protein aggregation and pathogenesis. Cytosolic accumulation of prion protein seems to be neurotoxic and might occur when proteasome function is compromised and quality control is overwhelmed. These findings are discussed in the light of other studies linking proteasome inhibition and neurodegeneration.
authors.affiliation: Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases/NIH, Hamilton, MT 59840, USA. ddimcheff@nih.gov
authors.forename: Derek E
authors.initials: DE
authors.lastname: Dimcheff
authors.forename: John L
authors.initials: JL
authors.lastname: Portis
authors.forename: Byron
authors.initials: B
authors.lastname: Caughey
mesh_terms: D000818:Animals; D003546:Cysteine Endopeptidases / Q000378:metabolism; D003600:Cytosol / Q000378:metabolism; D006801:Humans; D009097:Multienzyme Complexes / Q000378:metabolism; D009410:Nerve Degeneration / Q000378:metabolism / Q000503:physiopathology; D009474:Neurons / Q000378:metabolism* / Q000473:pathology; D018621:PrPC Proteins / Q000378:metabolism*; D018620:PrPSc Proteins / Q000378:metabolism*; D017096:Prion Diseases / Q000378:metabolism* / Q000503:physiopathology; D046988:Proteasome Endopeptidase Complex; D017510:Protein Folding; D021381:Protein Transport / Q000502:physiology
chemical_list: D009097:Multienzyme Complexes; D018621:PrPC Proteins; D018620:PrPSc Proteins; D003546:Cysteine Endopeptidases; D046988:Proteasome Endopeptidase Complex