8
May 2003
Muscle degeneration: Membrane repair defect in muscular dystrophy
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In some muscular dystrophies, muscle degeneration is due to a defect in the structural stability of the sarcolemma, the thin membrane enclosing the muscle fibres. Now comes evidence that disruption of membrane repair machinery also causes muscular dystrophy. Mutations in the gene for the protein dysferlin cause limb-girdle muscular dystrophy type 2B or Miyoshi myopathy. Targeted gene disruption in mice (cover inset) shows that dysferlin is a component of an active membrane repair process in skeletal muscle, and that disruption of this process produces the signs of muscular dystrophy. Upregulation of dysferlin expression could be a way of reducing severity of muscular dystrophies.
Defective membrane repair in dysferlin-deficient muscular
dystrophy
DIMPLE BANSAL, KATSUYA MIYAKE, STEVEN
S. VOGEL, S�VERINE GROH, CHIEN-CHANG CHEN, ROGER WILLIAMSON, PAUL L. MCNEIL &
KEVIN P. CAMPBELL
Nature 423, 168–172 (2003); doi:10.1038/nature01573
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Cell biology: Patches for wounded muscle
JULIET A. ELLIS
The stresses and strains imposed on certain
cells mean that their membranes require constant repair. Study of the damage that
affects muscle membranes reveals a new component of the repair process.
Nature
423, 129–131 (2003); doi:10.1038/423129a
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