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
- https://doi.org/10.1002/mus.880181007
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.This publication has 18 references indexed in Scilit:
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