NR AKKC
AU Sarkozi,E.; Askanas,V.; Engel,W.K.
TI Abnormal accumulation of prion protein mRNA in muscle fibers of patients with sporadic inclusion-body myositis and hereditary inclusion-body myopathy
QU American Journal of Pathology 1994 Dec; 145(6): 1280-4
KI Am J Pathol. 1994 Dec;145(6):1261-4. PMID: 7992830
PT journal article
AB Sporadic inclusion-body myositis is the most common progressive muscle disease of older patients. The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6 to 10-nm fibrils, both resembling those of Alzheimer brain, and Congo-red positivity. Hereditary inclusion-body myopathy designates patients cytopathologically similar but without inflammation. In both muscle diseases, prion, and several proteins characteristic of Alzheimer brain - eg, beta-amyloid protein and hyperphosphorylated tau (which normally are expressed mainly in neurons), and apolipoprotein E - are abnormally accumulated in vacuolated muscle fibers, by unknown mechanisms. We now demonstrate in both muscle diseases that prion mRNA is strongly expressed in the vacuolated muscle fibers, which suggests that their accumulated prion protein results, at least partly, from increased gene expression. This, to our knowledge, is the first demonstration of abnormally increased prion mRNA in human disease. Another novel finding is the increased prion mRNA in human muscle macrophages, and both increased prion protein and prion mRNA in regenerating muscle fibers. The latter indicates that prion may play a role in human muscle development.
IN In von der bei älteren Menschen häufigen Einschlußkörper-Myositis betroffenen ausgehöhlten Muskelfasern wurden 6-10 nm lange Kongorot-positive Fibrillen gefunden. Es gibt verschiedene entzündliche, aber auch eine erbliche Form ohne zelluläre Entzündungsreaktion. In beiden Typen wurde eine verstärkte Expression des Prionproteins in den betroffenen Muskelfasern, regenerierenden Muskelzellen und Muskelmakrophagen gefunden.
ZR 27
MH Adult; Aged; Human; Immunohistochemistry; In Situ Hybridization; Inclusion Bodies/*ultrastructure; Middle Age; Muscle Fibers/*metabolism; Muscular Diseases/metabolism/*pathology; Myositis/*metabolism/*pathology; Prions/*genetics/metabolism; RNA, Messenger/*metabolism; Support, Non-U.S. Gov't
AD Department of Neurology, University of Southern California School of Medicine, Los Angeles.
SP englisch
PO USA