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

Details

Serval ID
serval:BIB_EC0F9B83FD27
Type
Article: article from journal or magazin.
Collection
Publications
Title
Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.
Journal
Proceedings of the National Academy of Sciences of the United States of America
Author(s)
Han R., Kanagawa M., Yoshida-Moriguchi T., Rader E.P., Ng R.A., Michele D.E., Muirhead D.E., Kunz S., Moore S.A., Iannaccone S.T., Miyake K., McNeil P.L., Mayer U., Oldstone M.B., Faulkner J.A., Campbell K.P.
ISSN
1091-6490 (Electronic)
ISSN-L
0027-8424
Publication state
Published
Issued date
2009
Volume
106
Number
31
Pages
12573-12579
Language
english
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.
Keywords
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
Pubmed
Web of science
Open Access
Yes
Create date
17/04/2013 18:02
Last modification date
20/08/2019 17:14
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