Ubiquitin and beta-amyloid-protein in inclusion body myositis (IBM), familial IBM-like disorder and oculopharyngeal muscular dystrophy: an immunocytochemical study.
We used immunocytochemistry to identify ubiquitin and beta-amyloid-protein in muscle biopsies from patients with three neuromuscular disorders characterized by the presence of rimmed vacuoles in muscle fibres: inclusion body myositis (IBM), familial IBM-like disorder and oculopharyngeal muscular dystrophy (OPMD). Labelling with anti-ubiquitin antibodies was observed in all three diseases, but it was frequent in IBM, less common in familial IBM-like disorder and rare in OPMD. This labelling is thought to correspond to the presence of IBM-type filaments (16-18 nm in external diameter) which are characteristic but not specific for IBM or familial IBM-like disorder, as they may also occur in other diseases including OPMD. Labelling with anti-beta-amyloid-protein antibody was seen in a few fibres in IBM but not in the other two conditions. The structures labelled with this antibody have yet to be determined. Labelling with anti-ubiquitin or anti-beta-amyloid-protein antibodies was not correlated with the presence of acid phosphatase activity.
Leclerc
A
A
INSERM U. 153, Paris, France.
Tomé
F M
FM
Fardeau
M
M
D000328:Adult; D000368:Aged; D016229:Amyloid beta-Peptides / Q000032:analysis*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000473:pathology*; D009136:Muscular Dystrophies / Q000473:pathology*; D009220:Myositis / Q000473:pathology*; D009468:Neuromuscular Diseases / Q000473:pathology*; D014452:Ubiquitins / Q000032:analysis*; D014617:Vacuoles / Q000473:pathology / Q000648:ultrastructure
D016229:Amyloid beta-Peptides; D014452:Ubiquitins