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Mitochondrial abnormalities in inclusion-body myositis

From the Departments of Pathology (A.O., A.-R.M., L.J., M.O., G.K.) and Neurology (C.L.), Sahlgrenska University Hospital, Göteborg, Sweden.

Address correspondence and reprint requests to Dr. Anders Oldfors, Department of Pathology, Sahlgrenska University Hospital, S-413 45 Göteborg, Sweden; e-mail: anders.oldfors{at}pathology.gu.se

Mitochondrial changes are frequently encountered in sporadic inclusion-body myositis (s-IBM). Cytochrome c oxidase (COX)-deficient muscle fibers and large-scale mitochondrial DNA (mtDNA) deletions are more frequent in s-IBM than in age-matched controls. COX deficient muscle fibers are due to clonal expansion of mtDNA deletions and point mutations in segments of muscle fibers. Such segments range from 75 µm to more than 1,000 µm in length. Clonal expansion of the 4977 bp "common deletion" is a frequent cause of COX deficient muscle fiber segments, but many other deletions also occur. The deletion breakpoints cluster in a few regions that are similar to what is found in human mtDNA deletions in general. Analysis in s-IBM patients of three nuclear genes associated with multiple mtDNA deletions, POLG1, ANT1 and C10orf2, failed to demonstrate any mutations. In s-IBM patients with high number of COX-deficient fibers, the impaired mitochondrial function probably contribute to muscle weakness and wasting. Treatment that has positive effects in mitochondrial myopathies may be tried also in s-IBM.

This article was previously published in electronic format as an Expedited E-Pub at www.neurology.org.

Supported by grants from the Swedish Medical Research Council (Proj. No 7122), King Gustav V 80th Anniversary Fund, the Swedish Rheumatism Association, and the Göteborg Medical Society.

Disclosure: The authors report no conflicts of interest.

Received June 22, 2005. Accepted in final form October 14, 2005.

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