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Neurology 1999;53:2184
© 1999 American Academy of Neurology
Brief Communications

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

Janis McFerrin, BS, W. King Engel, MD and Valerie Askanas, MD, PhD

From the USC Neuromuscular Center, Department of Neurology, University of Southern California Keck School of Medicine, Good Samaritan Hospital, Los Angeles, CA.

Address correspondence and reprint requests to Dr. Valerie Askanas, USC Neuromuscular Center, 637 South Lucas Avenue, Los Angeles, CA 90017-1912; e-mail: askanas{at}hsc.usc.edu

Cultured muscle fibers from patients with sporadic inclusion-body myositis (s-IBM), similar to normal control muscle fibers, 1) did not have ß-amyloid precursor protein (ßAPP) 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 ßAPP 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.

Key words: Inclusion body myositis—ß-Amyloid precursor protein—Cultured muscle fibers—Innervation.




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[Abstract] [Full Text] [PDF]


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