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

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.
Authors	D Angoli, P Corona, R Baresi, M Mora, E Wanke
Journal	FEBS letters (FEBS Lett) Vol. 408 Issue 3 Pg. 341-4 (May 26 1997) ISSN: 0014-5793 [Print] England
PMID	9188790 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	Cytoskeletal Proteins DAG1 protein, human Laminin Membrane Glycoproteins Receptors, Laminin laminin alpha 2 Dystroglycans laminin A
Topics	Animals Cell Adhesion Cells, Cultured Cytoskeletal Proteins (physiology) Dystroglycans Extracellular Matrix (physiology) Humans Laminin (physiology) Membrane Glycoproteins (physiology) Mice Mice, Inbred mdx Muscle Fibers, Skeletal (physiology) Muscle, Skeletal (physiology, physiopathology) Muscular Dystrophies (physiopathology) Muscular Dystrophy, Animal (genetics, physiopathology) Receptors, Laminin (physiology) Reference Values Stress, Mechanical X Chromosome

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