Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration.

Abstract:

:Striated muscle-specific disruption of the dystroglycan (DAG1) gene results in loss of the dystrophin-glycoprotein complex in differentiated muscle and a remarkably mild muscular dystrophy with hypertrophy and without tissue fibrosis. We find that satellite cells, expressing dystroglycan, support continued efficient regeneration of skeletal muscle along with transient expression of dystroglycan in regenerating muscle fibers. We demonstrate a similar phenomenon of reexpression of functional dystroglycan in regenerating muscle fibers in a mild form of human muscular dystrophy caused by disruption of posttranslational dystroglycan processing. Thus, maintenance of regenerative capacity by satellite cells expressing dystroglycan is likely responsible for mild disease progression in mice and possibly humans. Therefore, inadequate repair of skeletal muscle by satellite cells represents an important mechanism affecting the pathogenesis of muscular dystrophy.

journal_name

Cell

journal_title

Cell

authors

Cohn RD,Henry MD,Michele DE,Barresi R,Saito F,Moore SA,Flanagan JD,Skwarchuk MW,Robbins ME,Mendell JR,Williamson RA,Campbell KP

doi

10.1016/s0092-8674(02)00907-8

subject

Has Abstract

pub_date

2002-09-06 00:00:00

pages

639-48

issue

5

eissn

0092-8674

issn

1097-4172

pii

S0092867402009078

journal_volume

110

pub_type

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