Strong immunoreactivity of ?1 co-localizes with ?-amyloid protein and ubiquitin in vacuolated muscle fibers of inclusion-body myositis

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Abstract
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.