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ZFIN ID: ZDB-PUB-010912-28
Dystrophin in adult zebrafish muscle
Chambers, S.P., Dodd, A., Overall, R., Sirey, T., Lam, L.T., Morris, G.E., and Love, D.R.
Date: 2001
Source: Biochemical and Biophysical Research Communications   286(3): 478-483 (Journal)
Registered Authors: Chambers, Steve, Dodd, Andrew, Love, Donald R.
Keywords: disease modelling; dystrophin; muscular dystrophy; syntrophin domain; zebrafish
MeSH Terms:
  • Amino Acid Sequence
  • Animals
  • Antibodies, Monoclonal/immunology
  • Dystrophin/genetics*
  • Dystrophin/immunology
  • Dystrophin/metabolism
  • HeLa Cells
  • Humans
  • Membrane Proteins/genetics
  • Membrane Proteins/metabolism*
  • Molecular Sequence Data
  • Muscle Proteins/genetics
  • Muscle Proteins/metabolism*
  • Muscle, Skeletal/metabolism*
  • Phylogeny
  • Sequence Homology, Amino Acid
  • Zebrafish/genetics*
  • Zebrafish/metabolism*
  • Zebrafish Proteins*
PubMed: 11511083 Full text @ Biochem. Biophys. Res. Commun.
ABSTRACT
Mutations in the human dystrophin gene are implicated in the fatal muscle wasting disease Duchenne Muscular Dystrophy (DMD). This gene expresses a sarcolemmal-associated protein that is evolutionarily conserved, underpinning its important role in the architecture of muscle. In terms of DMD modelling, the mouse has served as a suitable vertebrate species but the pathophysiology of the disease in the mouse does not entirely mimic human DMD. We have examined the zebrafish in order to expand the repertoire of vertebrate species for muscle disease modelling, and to dissect further the functional interactions of dystrophin. We report here the identification of an apparent zebrafish orthologue of the human dystrophin gene that expresses a 400-kDa protein that is localised to the muscle membrane surface. These data suggest that the zebrafish may prove to be a beneficial vertebrate model to examine the role and functional interactions of dystrophin in disease and development.
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