Biology of the Striated Muscle Dystrophin-Glycoprotein Complex

James M. Ervasti, Kevin J. Sonnemann

Research output: Chapter in Book/Report/Conference proceedingChapter

110 Scopus citations

Abstract

Since its first description in 1990, the dystrophin-glycoprotein complex has emerged as a critical nexus for human muscular dystrophies arising from defects in a variety of distinct genes. Studies in mammals widely support a primary role for the dystrophin-glycoprotein complex in mechanical stabilization of the plasma membrane in striated muscle and provide hints for secondary functions in organizing molecules involved in cellular signaling. Studies in model organisms confirm the importance of the dystrophin-glycoprotein complex for muscle cell viability and have provided new leads toward a full understanding of its secondary roles in muscle biology.

Original languageEnglish (US)
Title of host publicationA Survey of Cell Biology
PublisherAcademic Press Inc.
Pages191-225
Number of pages35
ISBN (Print)9780123743329
DOIs
StatePublished - 2008

Publication series

NameInternational Review of Cytology
Volume265
ISSN (Print)0074-7696

Bibliographical note

Funding Information:
The authors thank members of the Ervasti laboratory for their helpful feedback on this review and particularly Michele Jaeger for providing images in Fig. 5.1 . Supported by grants from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (AR042423 and AR049899), the Muscular Dystrophy Association, the Foundation to Eradicate Duchenne, the Nash Avery Foundation, and Charley’s Fund.

Keywords

  • Dystrobrevin
  • Dystroglycan
  • Dystrophin
  • Sarcoglycan
  • Sarcospan
  • Syntrophin

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