Dystrophin-positive fibers in Duchenne dystrophy: Origin and correlation to clinical course

M. Fanin, G. A. Danieli, M. Cadaldini, M. Miorin, L. Vitiello, C. Angelini

Research output: Contribution to journalArticlepeer-review

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 languageEnglish
Pages (from-to)1115-1120
Number of pages6
JournalMuscle and Nerve
Volume18
Issue number10
DOIs
Publication statusPublished - 1995

Keywords

  • Duchenne muscular dystrophy
  • duplication
  • dystrophin
  • reverted fibers

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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