NR AUBZ
AU Peden,A.H.; Ritchie,D.L.; Head,M.W.; Ironside,J.W.
TI Detection and localization of PrPsc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease
QU American Journal of Pathology 2006 Mar; 168(3): 927-35
PT journal article
AB Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that the pathogenic prion protein PrPsc can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. However, a recent study using a high-sensitivity Western blotting technique revealed low levels of PrPsc in skeletal muscle from a quarter of Swiss patients with sporadic CJD (sCJD). This posed the question of whether PrPsc in muscle could also be detected in vCJD, sCJD, and iatrogenic (iCJD) patients from other populations. Therefore, we have used the same high-sensitivity Western blotting technique, in combination with paraffin-embedded tissue blotting, to screen for PrPsc in muscle tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom. These techniques identified muscle PrPsc in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients. Paraffin-embedded tissue blotting analysis showed PrPsc in skeletal muscle in localized anatomical structures that had the morphological and immunohistochemical characteristics of nerve fibers. The detection of PrPsc in muscle tissue from all forms of CJD indicates the possible presence of infectivity in these tissues, suggesting important implications for assessing the potential risk of iatrogenic spread via contaminated surgical instruments.
MH Animals; Creutzfeldt-Jakob Syndrome/*diagnosis/genetics; Disease Models, Animal; Female; Humans; *Iatrogenic Disease; Male; Muscle, Skeletal/*chemistry/innervation/pathology; Myocardium/chemistry; Nerve Fibers/chemistry; PrPsc Proteins/*analysis; Research Support, Non-U.S. Gov't
AD Alexander H. Peden (a.peden@ed.ac.uk), Diane L. Ritchie, Mark W. Head (m.w.head@ed.ac.uk), James W. Ironside (james.ironside@ed.ac.uk), 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
SP englisch
PO USA