title: Preclinical deposition of pathological prion protein in muscle of experimentally infected primates.
abstract: Prion diseases are transmissible fatal neurodegenerative disorders affecting humans and animals. A central step in disease progression is the accumulation of a misfolded form (PrP(Sc)) of the host encoded prion protein (PrP(C)) in neuronal and non-neuronal tissues. The involvement of peripheral tissues in preclinical states increases the risk of accidental transmission. On the other hand, detection of PrP(Sc) in non-neuronal easy-accessible compartments such as muscle may offer a novel diagnostic tool. Primate models have proven invaluable to investigate prion diseases. We have studied the deposition of PrP(Sc) in muscle and central nervous system of rhesus monkeys challenged with sporadic Creutzfeldt-Jakob disease (sCJD), variant CJD (vCJD) and bovine spongiform encephalopathy (BSE) in preclinical and clinical stage using biochemical and morphological methods. Here, we show the preclinical presence of PrP(Sc) in muscle and central nervous system of rhesus monkeys experimentally infected with vCJD.
authors.affiliation: Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Krasemann
authors.forename: Melanie
authors.initials: M
authors.lastname: Neumann
authors.forename: Markus
authors.initials: M
authors.lastname: Geissen
authors.forename: Walter
authors.initials: W
authors.lastname: Bodemer
authors.forename: Franz-Josef
authors.initials: FJ
authors.lastname: Kaup
authors.forename: Walter
authors.initials: W
authors.lastname: Schulz-Schaeffer
authors.forename: Nathalie
authors.initials: N
authors.lastname: Morel
authors.forename: Adriano
authors.initials: A
authors.lastname: Aguzzi
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
mesh_terms: D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism; D008253:Macaca mulatta; D009132:Muscles / Q000378:metabolism*; D009206:Myocardium / Q000378:metabolism; D018620:PrPSc Proteins / Q000378:metabolism*; D017096:Prion Diseases / Q000378:metabolism* / Q000473:pathology; D014059:Tongue / Q000378:metabolism
chemical_list: D018620:PrPSc Proteins