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 language | English (US) |
|---|---|
| Title of host publication | A Survey of Cell Biology |
| Publisher | Academic Press Inc. |
| Pages | 191-225 |
| Number of pages | 35 |
| ISBN (Print) | 9780123743329 |
| DOIs | |
| State | Published - 2008 |
Publication series
| Name | International Review of Cytology |
|---|---|
| Volume | 265 |
| 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