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, 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.
McFerrin
J
J
USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles 90017-1912, USA.
Engel
W K
WK
Askanas
V
V
D000368:Aged; D016564:Amyloid beta-Protein Precursor / Q000378:metabolism*; D000818:Animals; D046508:Culture Techniques; D005727:Ganglia, Spinal / Q000473:pathology; D006801:Humans; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000473:pathology*; D018482:Muscle, Skeletal / Q000294:innervation* / Q000473:pathology; D018979:Myositis, Inclusion Body / Q000473:pathology*; D051381:Rats; D013116:Spinal Cord / Q000473:pathology
D016564:Amyloid beta-Protein Precursor