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Acta Neuropathol. 2006 Aug;112(2):185-93. Epub 2006 Jun 21.

Rimmed vacuoles with beta-amyloid and tau protein deposits in the muscle of children with hereditary myopathy.

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Neuromuscular Unit MRC, Polish Academy of Science, Pawińskiego 5, 02-106, Warsaw, Poland.


We investigated whether beta-amyloid and tau protein are involved in the formation of inclusion body myositis (IBM)-like inclusions found in children with rimmed vacuoles and congenitally affected muscles. We immunostained muscle biopsy specimens from four children and one 18-year-old boy with congenital myopathy containing rimmed vacuoles and IBM-like inclusions with antibodies against beta-amyloid, tau protein and ubiquitin. Focal accumulations of both beta-amyloid and phosphorylated tau coexisted with tubulofilamentous structures in all cases. Our studies demonstrate for the first time that the full morphological phenotype of IBM including beta-amyloid and tau protein deposits may also develop in children, and that congenital, probably genetic, muscle defects may lead to abnormal protein aggregation in IBM-like inclusions.

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