Journal article
Disruption of Dag1 in Differentiated Skeletal Muscle Reveals a Role for Dystroglycan in Muscle Regeneration
Cell (Cambridge), Vol.110(5), pp.639-648
2002
DOI: 10.1016/S0092-8674(02)00907-8
PMID: 12230980
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.
Details
- Title: Subtitle
- Disruption of Dag1 in Differentiated Skeletal Muscle Reveals a Role for Dystroglycan in Muscle Regeneration
- Creators
- Ronald D Cohn - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USAMichael D Henry - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USADaniel E Michele - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USARita Barresi - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USAFumiaki Saito - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USASteven A Moore - Department of Pathology, Iowa City, IA 52242 USAJason D Flanagan - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USAMark W Skwarchuk - Free Radical and Radiation Biology Program, Department of Radiation Oncology, Holden Comprehensive Cancer Center, Iowa City, IA 52242 USAMichael E Robbins - Free Radical and Radiation Biology Program, Department of Radiation Oncology, Holden Comprehensive Cancer Center, Iowa City, IA 52242 USAJerry R Mendell - Department of Neurology, Ohio State University Hospital, Columbus, OH 43210 USARoger A Williamson - Department of Obstetrics and Gynecology, College of Medicine, University of Iowa, Iowa City, IA 52242 USAKevin P Campbell - Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242 USA
- Resource Type
- Journal article
- Publication Details
- Cell (Cambridge), Vol.110(5), pp.639-648
- Publisher
- Elsevier Inc
- DOI
- 10.1016/S0092-8674(02)00907-8
- PMID
- 12230980
- ISSN
- 0092-8674
- eISSN
- 1097-4172
- Language
- English
- Date published
- 2002
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Pathology; Iowa Neuroscience Institute; Radiation Oncology; Obstetrics and Gynecology; Urology
- Record Identifier
- 9984047728002771
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