Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan

Proc Natl Acad Sci U S A. 2009 Aug 4;106(31):12573-9. doi: 10.1073/pnas.0906545106. Epub 2009 Jul 24.

Abstract

Skeletal muscle basal lamina is linked to the sarcolemma through transmembrane receptors, including integrins and dystroglycan. The function of dystroglycan relies critically on posttranslational glycosylation, a common target shared by a genetically heterogeneous group of muscular dystrophies characterized by alpha-dystroglycan hypoglycosylation. Here we show that both dystroglycan and integrin alpha7 contribute to force-production of muscles, but that only disruption of dystroglycan causes detachment of the basal lamina from the sarcolemma and renders muscle prone to contraction-induced injury. These phenotypes of dystroglycan-null muscles are recapitulated by Large(myd) muscles, which have an intact dystrophin-glycoprotein complex and lack only the laminin globular domain-binding motif on alpha-dystroglycan. Compromised sarcolemmal integrity is directly shown in Large(myd) muscles and similarly in normal muscles when arenaviruses compete with matrix proteins for binding alpha-dystroglycan. These data provide direct mechanistic insight into how the dystroglycan-linked basal lamina contributes to the maintenance of sarcolemmal integrity and protects muscles from damage.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Animals
  • Basement Membrane / physiology*
  • Binding Sites
  • Dystroglycans / chemistry
  • Dystroglycans / physiology*
  • Glycosylation
  • Integrins / physiology
  • Laminin / chemistry
  • Laminin / physiology*
  • Lymphocytic choriomeningitis virus
  • Mice
  • Muscular Dystrophy, Animal / etiology
  • Sarcolemma / physiology*

Substances

  • Integrins
  • Laminin
  • Dystroglycans
  • integrin alpha7beta1