Journal Article

Expression of the dystrophin isoform Dp71 in differentiating human fetal myogenic cultures

Christine N. Tennyson, Ghassan Y. Dally, Peter N. Ray and Ronald G. Worton

in Human Molecular Genetics

Volume 5, issue 10, pages 1559-1566
Published in print October 1996 | ISSN: 0964-6906
e-ISSN: 1460-2083 | DOI:
Expression of the dystrophin isoform Dp71 in differentiating human fetal myogenic cultures

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The dystrophin gene defective in Duchenne muscular dystrophy (DMD) is extreme in size and complexity with several promoters which direct expression of different isoforms in different tissues. In contrast with adult skeletal muscle which expresses 427 kDa dystrophin, fetal muscle tissue expresses the 71 kDa ubiquitous isoform Dp71 as well as 427 kDa muscle dystrophin. To examine Dp71 expression in fetal muscle further, we have monitored its expression pattern in differentiating myogenic cultures of human fetal muscle origin. The presence of transcripts initiated from the Dp71 promoter was demonstrated by quantitative RT-PCR. The level of transcript expressed from the Dp71 promoter did not change significantly during myogenic differentiation, consistent with the housekeeping nature of the promoter. Measurements to determine the stability of the Dp71 mRNA indicated that it has a half-life of 20 h and, therefore, is somewhat more stable than the larger 14 kb muscle dystrophin mRNA (t½ = 16 h). In contrast with the constant level of Dp71 transcript during myogenic differentiation, the level of Dp71 protein increased significantly, perhaps due to changes in translation efficiency or protein stability. These results demonstrate expression and posttranscriptional upregulation of Dp71 in human fetal myogenic cultures.

Journal Article.  6738 words.  Illustrated.

Subjects: Genetics and Genomics

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