NR AAUV
AU Askanas,V.; Engel,W.K.; Mirabella,M.
TI Idiopathic inflammatory myopathies: inclusion-body myositis, polymyositis, and dermatomyositis.
QU Current Opinion in Neurology 1994 Oct; 7(5): 448-56
PT journal article; review; review, tutorial
AB In this review, the main emphasis is on new advances concerning sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Polymyositis and dermatomyositis are reviewed briefly. Hypotheses are presented regarding the possible cause and significance of abnormally accumulated beta-amyloid protein, two other epitopes of beta-amyloid precursor protein, hyperphosphorylated tau, alpha 1-antichymotrypsin, ubiquitin, and prion protein in sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Because most of those proteins are also accumulated at the neuromuscular junction, "junctionalization" of other muscle fiber nuclei is a possibility. Attention is given to the fact that vacuolated muscle fibers in hereditary inclusion-body myopathy may represent early changes because they are virtually free of congophilic amyloid deposit but, like sporadic inclusion-body myositis, contain large accumulations of beta-amyloid protein and prion.
ZR 77
MH Aged; Amyloid beta-Protein/genetics; Amyloid beta-Protein Precursor/genetics; Dermatomyositis/*genetics/pathology; Diagnosis, Differential; Human; Inclusion Bodies/*pathology; Middle Age; Muscles/pathology; Myositis/*genetics/pathology; Polymyositis/*genetics/pathology; Prions/genetics; RNA, Messenger/genetics; Tissue Culture
AD University of Southern California School of Medicine, Department of Neurology, Los Angeles 90017-1912.
SP englisch
PO USA