Expression of the Dystrophin Gene in Cultured Fibroblasts

Abstract : The dystrophin whose defect is responsible for Duchenne and Becker muscular dystrophies is present in muscle, brain and cerebellum. We describe here the detection of dystrophin in human cultured skin fibroblasts, L809 cells and murine 3T6 cell line. Dystrophin transcripts initiated at the muscle specific first exon can also be amplified by cDNA-PCR from various fibroblastic cells. The expression of the dystrophin gene in fibroblasts could account for some abnormalities observed in patient's fibroblast cultures.
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J.P. Hugnot, H. Gilgenkrantz, P. Chafey, M. Lambert, Eric Eveno, et al.. Expression of the Dystrophin Gene in Cultured Fibroblasts. Biochemical and Biophysical Research Communications, Elsevier, 1993, 192 (1), pp.69-74. ⟨10.1006/bbrc.1993.1382⟩. ⟨hal-02115517⟩



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