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.
Original language | English |
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Pages (from-to) | 1115-1120 |
Number of pages | 6 |
Journal | Muscle and Nerve |
Volume | 18 |
Issue number | 10 |
DOIs | |
Publication status | Published - 1995 |
Keywords
- Duchenne muscular dystrophy
- duplication
- dystrophin
- reverted fibers
ASJC Scopus subject areas
- Clinical Neurology
- Neuroscience(all)