An unusual variant of Becker muscular dystrophy
โ Scribed by Marianne De Visser; Egbert Bakker; Joep C. Defesche; Piet A. Bolhuis; Gert Jan Van Ommen
- Publisher
- John Wiley and Sons
- Year
- 1990
- Tongue
- English
- Weight
- 710 KB
- Volume
- 27
- Category
- Article
- ISSN
- 0364-5134
No coin nor oath required. For personal study only.
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Duchenne muscular dystrophy (DMD) is a progressive and lethal neuromuscular disorder caused by a defective gene on the X chromosome. There is no effective treatment and the biochemical defect is yet unknown. Mapping of the DMD locus to band Xp21 in the short arm of the X chromosome has given rise to
We studied by high-resolution immunofluorescence (HRI) and by confocal laser scanning optical microscopy (CLSOM) the costameric organization of dystrophin and vinculin at the surface membrane of muscle fibers from 4 young boys with Becker muscular dystrophy (BMD). By HRI, the surface membrane of nor