ZFIN ID: ZDB-PUB-050215-3
delta-Sarcoglycan is required for early zebrafish muscle organization
Guyon, J.R., Mosley, A.N., Jun, S.J., Montanaro, F., Steffen, L.S., Zhou, Y., Nigro, V., Zon, L.I., and Kunkel, L.M.
Date: 2005
Source: Experimental cell research 304(1): 105-115 (Journal)
Registered Authors: Guyon, Jeff, Kunkel, Louis M., Zhou, Yi, Zon, Leonard I.
Keywords: Muscular dystrophy; Limb girdle muscular dystrophy; Sarcoglycan; Zebrafish; Sarcolemmal membrane; Myosepta; DAPC
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*
PubMed: 15707578 Full text @ Exp. Cell Res.
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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.
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