@article{1436f4fae49d4bf2ad676389cf56242b,
title = "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.",
author = "Cohn, {Ronald D.} and Henry, {Michael D.} and Michele, {Daniel E.} and Rita Barresi and Fumiaki Saito and Moore, {Steven A.} and Flanagan, {Jason D.} and Skwarchuk, {Mark W.} and Robbins, {Michael E.} and Mendell, {Jerry R.} and Williamson, {Roger A.} and Campbell, {Kevin P.}",
note = "Funding Information: We would like to thank Reinhard F{\"a}ssler for his kind gifts of plasmids that we used for construction of the floxed allele. We also would like to thank C. Ronald Kahn for generously providing us with MCK-Cre mice. We would like to thank Sarah Lowen and all members of the Campbell laboratory for the critical reading of the manuscript, fruitful discussions, and supply of critical reagents. We would like to thank Steve Westra for technical assistance and Stuart Weinstein for help with obtaining muscle biopsy tissue. We thank the University of Iowa DNA Core Facility, which is supported in part by the Diabetes Endocrinology Research Center (NIH DK25295) and the University of Iowa, Roy J. and Lucille A. Carver College of Medicine. R.D.C. was supported by the Deutsche Forschungsgemeinschaft (Co 241-1). D.E.M. was supported by an HL07121 Cardiovascular Interdisciplinary Research Fellowship and a Biosciences Initiative Research Fellowship. This work was also supported by the Muscular Dystrophy Association (K.P.C. and S.A.M.). K.P.C. is an Investigator of the Howard Hughes Medical Institute.",
year = "2002",
month = sep,
day = "6",
doi = "10.1016/S0092-8674(02)00907-8",
language = "English (US)",
volume = "110",
pages = "639--648",
journal = "Cell",
issn = "0092-8674",
publisher = "Cell Press",
number = "5",
}