Laminin-alpha2 but not -alpha1-mediated adhesion of human (Duchenne) and murine (mdx) dystrophic myotubes is seriously defective

FEBS Letters
D AngoliE Wanke

Abstract

It has been suggested that alpha-dystroglycan links the dystrophin-associated protein complex and extracellular matrix and that the absence of dystrophin and alpha-dystroglycan in Duchenne muscular dystrophy (DMD) may lead to the breakdown of this linkage. In the present study, myotubes from DMD patients and murine X-linked muscular dystrophic mice (mdx) were used to measure their adhesive force to the physiological laminin-alpha2 substrate, and it was found that the dystrophic myotubes were selectively unable to sustain adhesion. However, normal and dystrophic myotubes attached equally well to the laminin-alpha1 substrate. As far as we know, this is the first experimental evidence that the absence of dystrophin causes the complete loss of a still unknown laminin-alpha2-dependent adhesion force, therefore suggesting that the primary consequence of Duchenne dystrophy consists of the loss of an authentic mechanical linkage at the level of the alpha-dystroglycan/basal lamina interface.

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Citations

Jun 15, 1999·Matrix Biology : Journal of the International Society for Matrix Biology·M Aumailley, P Rousselle
Oct 3, 1998·Journal of Anatomy·M Aumailley, N Smyth
May 27, 2014·Materials Science & Engineering. C, Materials for Biological Applications·B CodanO Sbaizero
Jan 30, 2004·Biophysical Journal·Maureen A GriffinDennis E Discher

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