title: Could inhibition of the proteasome cause mad cow disease?
abstract: The proteasome is the cellular machinery responsible for the degradation of normal and misfolded proteins. Inhibitors of the proteasome are being evaluated as therapeutic agents and recent work suggests that such inhibition might promote the neurotoxic properties of the prion protein (the causative agent of mad cow disease) and its conformational conversion to the infectious form, thus raising the question as to whether proteasome inhibitors might facilitate the development of prion diseases.
authors.affiliation: School of Biochemistry and Molecular Biology, University of Leeds, Leeds LS2 9JT, UK. n.m.hooper@leeds.ac.uk
authors.forename: Nigel M
authors.initials: NM
authors.lastname: Hooper
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000737:chemistry / Q000378:metabolism*; D000818:Animals; D002417:Cattle; D003546:Cysteine Endopeptidases / Q000737:chemistry / Q000378:metabolism*; D003600:Cytosol / Q000378:metabolism*; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism* / Q000635:transmission; D004721:Endoplasmic Reticulum / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D009097:Multienzyme Complexes / Q000037:antagonists & inhibitors* / Q000737:chemistry / Q000378:metabolism*; D011480:Protease Inhibitors / Q000378:metabolism*; D046988:Proteasome Endopeptidase Complex; D011487:Protein Conformation; D017510:Protein Folding; D021381:Protein Transport
chemical_list: C107991:ABCB6 protein, human; D018528:ATP-Binding Cassette Transporters; D009097:Multienzyme Complexes; D011480:Protease Inhibitors; D003546:Cysteine Endopeptidases; D046988:Proteasome Endopeptidase Complex