Human Molecular Genetics, Vol 5, 1559-1566, Copyright © 1996 by Oxford University Press
CN Tennyson, GY Dally, PN Ray and RG Worton
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 (t1/2 = 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.
ARTICLES
Expression of the dystrophin isoform Dp71 in differentiating human fetal myogenic cultures
Department of Molecular and Medical Genetics, University of Toronto, Ontario, Canada.
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