Dystrophin-positive fibers in Duchenne dystrophy: origin and correlation to clinical course
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...
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Published in | Muscle & nerve Vol. 18; no. 10; p. 1115 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
United States
01.10.1995
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Subjects | |
Online Access | Get more information |
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Summary: | 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 microns. 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. |
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ISSN: | 0148-639X |
DOI: | 10.1002/mus.880181007 |