Delta-sarcoglycan is required for early zebrafish muscle organization

Exp Cell Res. 2005 Mar 10;304(1):105-15. doi: 10.1016/j.yexcr.2004.10.032. Epub 2004 Dec 8.

Abstract

Mutations in sarcoglycans (alpha-, beta-, gamma-, and delta-) have been linked with limb girdle muscular dystrophy (LGMD) types 2C-F in humans. We have cloned the zebrafish orthologue encoding delta-sarcoglycan and mapped the gene to linkage group 21. The predicted zebrafish delta-sarcoglycan protein is highly homologous with its human orthologue including conservation of two of the three predicted glycosylation sites. Like other members of the dystrophin-associated protein complex (DAPC), delta-sarcoglycan localizes to the sarcolemmal membrane of the myofiber in adult zebrafish, but is more apparent at the myosepta in developing embryos. Zebrafish embryos injected with morpholinos against delta-sarcoglycan were relatively inactive at 5 dpf, their myofibers were disorganized, and swim bladders uninflated. Immunohistochemical and immunoblotting experiments show that delta-, beta-, and gamma-sarcoglycans were all downregulated in the morphants, whereas dystrophin expression was unaffected. Whereas humans lacking delta-sarcoglycan primarily show adult phenotypes, our results suggest that delta-sarcoglycan plays a role in early zebrafish muscle development.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Base Sequence
  • Cloning, Molecular
  • Down-Regulation
  • Dystrophin / metabolism
  • Humans
  • Mice
  • Molecular Sequence Data
  • Muscle, Skeletal / cytology
  • Muscle, Skeletal / embryology*
  • Sarcoglycans / analysis
  • Sarcoglycans / genetics*
  • Sarcoglycans / physiology*
  • Sarcolemma / chemistry
  • Zebrafish / embryology*
  • Zebrafish Proteins / genetics*
  • Zebrafish Proteins / physiology*

Substances

  • Dystrophin
  • Sarcoglycans
  • Zebrafish Proteins