β-amyloid precursor protein mRNA is increased in inclusion-body myositis muscle

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Abstract
Vacuolated muscle fibers in muscle biopsies of 8 out of 8 inclusion body myositis (IBM) patients, including 2 hereditary patients, manifested increased mRNA for the β-amyloid precursor protein (βAPP) that contains Kunitz-type protease inhibitor motif. In affected fibers, increased βAPP-mRNA corresponded to abnormally accumulated βAPP immunoreactivity (including β-amyloid protein epitope). In normal human muscle fibers increased βAPP-mRNA was present only at the neuromuscular junctions. Our study (a) suggests that abnormally accumulated βAPP in IBM vacuolated fibers results, at least partly, from increased βAPP generation, and (b) provides the first demonstration of up-regulated βAPP-mRNA in pathologic human tissue other than brain of Alzheimer's disease and Down's syndrome.

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