Cultured inclusion-body myositis muscle fibers do not accumulate beta-amyloid precursor protein and can be innervated

Neurology. 1999 Dec 10;53(9):2184-7. doi: 10.1212/wnl.53.9.2184.

Abstract

Cultured muscle fibers from patients with sporadic inclusion-body myositis (s-IBM), similar to normal control muscle fibers, 1) did not have beta-amyloid precursor protein (betaAPP) immunoreactivity; and 2) became normally innervated, as evidenced by the following features: full cross-striation, continuous contraction, and acetylcholinesterase and acetylcholine receptors accumulated only at neuromuscular junctions. Thus, factors responsible for betaAPP accumulation and denervation-like changes in s-IBM muscle biopsies are not operative in the relatively short-term, non-aged, cultured s-IBM muscle fibers.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Aged
  • Amyloid beta-Protein Precursor / metabolism*
  • Animals
  • Culture Techniques
  • Ganglia, Spinal / pathology
  • Humans
  • Microscopy, Fluorescence
  • Middle Aged
  • Muscle Fibers, Skeletal / pathology*
  • Muscle, Skeletal / innervation*
  • Muscle, Skeletal / pathology
  • Myositis, Inclusion Body / pathology*
  • Rats
  • Spinal Cord / pathology

Substances

  • Amyloid beta-Protein Precursor