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Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease. | LitMetric

Detection and localization of PrPSc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease.

Am J Pathol

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.

Published: March 2006

AI Article Synopsis

  • Variant Creutzfeldt-Jakob disease (vCJD) is different from other prion diseases because the harmful protein PrP(Sc) is found more outside the nervous system, particularly in lymphoid tissues.
  • A new study has revealed that low levels of PrP(Sc) can also be detected in skeletal muscle tissues of some patients with sporadic CJD (sCJD).
  • Researchers found PrP(Sc) in muscle samples from various types of CJD patients, which raises concerns about the risk of spreading the disease through contaminated surgical tools.

Article Abstract

Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that the pathogenic prion protein PrP(Sc) 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 PrP(Sc) in skeletal muscle from a quarter of Swiss patients with sporadic CJD (sCJD). This posed the question of whether PrP(Sc) 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 PrP(Sc) in muscle tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom. These techniques identified muscle PrP(Sc) in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients. Paraffin-embedded tissue blotting analysis showed PrP(Sc) in skeletal muscle in localized anatomical structures that had the morphological and immunohistochemical characteristics of nerve fibers. The detection of PrP(Sc) 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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1606529PMC
http://dx.doi.org/10.2353/ajpath.2006.050788DOI Listing

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