title: Peripheral tissue involvement in sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative, and biochemical study.
abstract: Human prion diseases are rare fatal neurodegenerative conditions that occur as acquired, familial, or idiopathic disorders. A key event in their pathogenesis is the accumulation of an altered form of the prion protein, termed PrP(Sc), in the central nervous system. A novel acquired human prion disease, variant Creutzfeldt-Jakob disease, is thought to result from oral exposure to the bovine spongiform encephalopathy agent. This disease differs from other human prion diseases in its neurological, neuropathological, and biochemical phenotype. We have used immunohistochemistry and Western blot techniques to analyze the tissue distribution and biochemical properties of PrP(Sc) in peripheral tissues in a unique series of nine cases of variant Creutzfeldt-Jakob disease. We have compared this with the distribution and biochemical forms found in all of the major subtypes of sporadic Creutzfeldt-Jakob disease and in a case of iatrogenic Creutzfeldt-Jakob disease associated with growth hormone therapy. The results show that involvement of the lymphoreticular system is a defining feature of variant Creutzfeldt-Jakob disease, but that the biochemical isoform of PrP(Sc) found is influenced by the cell type in which it accumulates.
authors.affiliation: National Creutzfeldt-Jakob Disease Surveillance Unit and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom. m.w.head@ed.ac.uk
authors.forename: Mark W
authors.initials: MW
authors.lastname: Head
authors.forename: Diane
authors.initials: D
authors.lastname: Ritchie
authors.forename: Nadine
authors.initials: N
authors.lastname: Smith
authors.forename: Victoria
authors.initials: V
authors.lastname: McLoughlin
authors.forename: William
authors.initials: W
authors.lastname: Nailon
authors.forename: Sazia
authors.initials: S
authors.lastname: Samad
authors.forename: Stephen
authors.initials: S
authors.lastname: Masson
authors.forename: Matthew
authors.initials: M
authors.lastname: Bishop
authors.forename: Linda
authors.initials: L
authors.lastname: McCardle
authors.forename: James W
authors.initials: JW
authors.lastname: Ironside
mesh_terms: D015153:Blotting, Western; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D001923:Brain Chemistry; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007150:Immunohistochemistry; D020961:Lewy Body Disease / Q000473:pathology; D014066:Palatine Tonsil / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology; D018620:PrPSc Proteins / Q000737:chemistry* / Q000378:metabolism*
chemical_list: D018620:PrPSc Proteins