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Strong immunoreactivity of α1 co-localizes with β-amyloid protein and ubiquitin in vacuolated muscle fibers of inclusion-body myositis

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Summary

In 10 of 10 inclusion-body myositis (IBM) patients, including 1 hereditary case, vacuolated muscle fibers contained large or small cytoplasmic inclusions immunoreactive for (α1-ACT). All IBM muscle biopsies had characteristic cytoplasmic tubulo-filaments by electron microscopy. None of 17 control muscle biopsies contained the (α1-ACT) immunoreactive inclusions characteristic of IBM. In vacuolated muscle fibers, (α1-ACT) immunoreactive inclusions colocalized with β-amyloid protein and ubiquitin immunoreactivities. Our study provides the first demonstration of (α1-ACT) accumulations in abnormal human muscle, and it suggest that, as in Alzheimer's disease and Down's syndrome, (α1-ACT) may be involved in the pathogenesis of IBM.

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Authors and Affiliations

  1. USC Neuromuscular Center, Department of Neurology, University of Southern California School of Medicine, 637 S. Lucas Ave., 90017, Los Angeles, CA, USA

    Masako Bilak, Valerie Askanas & W. King Engel

Authors
  1. Masako Bilak
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  2. Valerie Askanas
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  3. W. King Engel
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Supported in part by the Norma Bard Research Fund. M.B. is a Research Postdoctoral Fellow

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Bilak, M., Askanas, V. & Engel, W.K. Strong immunoreactivity of α1 co-localizes with β-amyloid protein and ubiquitin in vacuolated muscle fibers of inclusion-body myositis. Acta Neuropathol 85, 378–382 (1993). https://doi.org/10.1007/BF00334447

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  • DOI: https://doi.org/10.1007/BF00334447

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