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

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, con...

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Bibliographic Details
Published inNeurology Vol. 53; no. 9; p. 2184
Main Authors McFerrin, J, Engel, W K, Askanas, V
Format Journal Article
LanguageEnglish
Published United States 10.12.1999
Subjects
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
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Summary: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.
ISSN:0028-3878
DOI:10.1212/wnl.53.9.2184