Ubiquitin and beta-amyloid-protein in inclusion body myositis (IBM), familial IBM-like disorder and oculopharyngeal muscular dystrophy: an immunocytochemical study

Neuromuscul Disord. 1993 Jul;3(4):283-91. doi: 10.1016/0960-8966(93)90021-b.

Abstract

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.

MeSH terms

  • Adult
  • Aged
  • Amyloid beta-Peptides / analysis*
  • Child, Preschool
  • Female
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies
  • Male
  • Middle Aged
  • Muscles / pathology*
  • Muscular Dystrophies / pathology*
  • Myositis / pathology*
  • Neuromuscular Diseases / pathology*
  • Ubiquitins / analysis*
  • Vacuoles / pathology
  • Vacuoles / ultrastructure

Substances

  • Amyloid beta-Peptides
  • Ubiquitins