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Analysis of Myogenic Markers in Duchenne's Muscular Dystrophy Cell Models
Author(s) -
Harnois Melissa,
Pincus Mark,
Powers Elaine,
Badarinarayana Vasu
Publication year - 2016
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.30.1_supplement.828.1
Subject(s) - myogenesis , duchenne muscular dystrophy , myocyte , biology , muscular dystrophy , immunohistochemistry , gene expression profiling , microbiology and biotechnology , gene expression , gene , genetics , immunology
The goal of the project was to compare the in vitro differentiation potential of Duchenne's Muscular Dystrophy (DMD) and non‐disease patient derived myoblasts. The differentiation of myoblasts into myotubes as well as the maturity of myotubes was assessed by morphology, immunohistochemistry (IHC) analysis of myotubes markers as well as transcript profiles of myogenesis markers. Human myoblasts derived from three non‐disease and three DMD patients were evaluated in multiple time‐course studies. Morphological evaluation as well as IHC analysis indicated that the DMD patient derived myoblasts have diminished capacity to differentiate and form mature myotubes. Gene expression profiling also revealed significantly reduced basal transcript levels of myogenic markers in DMD patient derived cells and impaired induction of these transcripts during differentiation. Support or Funding Information Sanofi Tucson Innovation Center, Oro Valley, AZ