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
Celljournal_title
Cellauthors
Cohn RD,Henry MD,Michele DE,Barresi R,Saito F,Moore SA,Flanagan JD,Skwarchuk MW,Robbins ME,Mendell JR,Williamson RA,Campbell KPdoi
10.1016/s0092-8674(02)00907-8subject
Has Abstractpub_date
2002-09-06 00:00:00pages
639-48issue
5eissn
0092-8674issn
1097-4172pii
S0092867402009078journal_volume
110pub_type
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