Dystrophin‐positive fibers in duchenne dystrophy: Origin and correlation to clinical course
- 1 October 1995
- journal article
- research article
- Published by Wiley in Muscle & Nerve
- Vol. 18 (10) , 1115-1120
Abstract
In 132 DMD muscle biopsies we investigated the presence of dystrophin‐positive fibers and the relationship of dystrophin immunohistochemical pattern to the clinical severity of the disease. Reverted fibers were detected in 37% of patients; their prevalence increased significantly in each biopsy with age of patients. We suggest that reversion occurs in satellite cells, when muscle differentiation is completed. The longitudinal extent of dystrophin‐positive domain spans a maximum length of 900 μm. No correlation was found between the presence of reverted fibers and the clinical severity of DMD, whereas a milder form of Duchenne dystrophy was observed in patients showing a faint reaction in all fibers. The occurrence of reverted fibers is independent of the type of gene mutation; however, a higher proportion of cases with reverted fibers was found among patients with gene duplications.© 1995 John Wiley & Sons, Inc.Keywords
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