The Dystrophin Glycoprotein Complex: Signaling Strength and Integrity for the Sarcolemma

Karen A. Lapidos, Rahul Kakkar, Elizabeth M. McNally*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

336 Scopus citations

Abstract

The dystrophin glycoprotein complex (DGC) is a specialization of cardiac and skeletal muscle membrane. This large multicomponent complex has both mechanical stabilizing and signaling roles in mediating interactions between the cytoskeleton, membrane, and extracellular matrix. Dystrophin, the protein product of the Duchenne and X-linked dilated cardiomyopathy locus, links cytoskeletal and membrane elements. Mutations in additional DGC genes, the sarcoglycans, also lead to cardiomyopathy and muscular dystrophy. Animal models of DGC mutants have shown that destabilization of the DGC leads to membrane fragility and loss of membrane integrity, resulting in degeneration of skeletal muscle and cardiomyocytes. Vascular reactivity is altered in response to primary degeneration in striated myocytes and arises from a vascular smooth muscle cell-extrinsic mechanism.

Original languageEnglish (US)
Pages (from-to)1023-1031
Number of pages9
JournalCirculation research
Volume94
Issue number8
DOIs
StatePublished - Apr 30 2004

Keywords

  • Cardiomyocyte
  • Dystrophin
  • Membrane
  • Sarcoglycan
  • Skeletal muscle

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Fingerprint Dive into the research topics of 'The Dystrophin Glycoprotein Complex: Signaling Strength and Integrity for the Sarcolemma'. Together they form a unique fingerprint.

Cite this