title: Dystrophin in adult zebrafish muscle.
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.
authors.affiliation: Molecular Genetics and Development Group, School of Biological Sciences, University of Auckland, Auckland, New Zealand.
authors.forename: S P
authors.initials: SP
authors.lastname: Chambers
authors.forename: A
authors.initials: A
authors.lastname: Dodd
authors.forename: R
authors.initials: R
authors.lastname: Overall
authors.forename: T
authors.initials: T
authors.lastname: Sirey
authors.forename: L T
authors.initials: LT
authors.lastname: Lam
authors.forename: G E
authors.initials: GE
authors.lastname: Morris
authors.forename: D R
authors.initials: DR
authors.lastname: Love
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D000911:Antibodies, Monoclonal / Q000276:immunology; D016189:Dystrophin / Q000235:genetics* / Q000276:immunology / Q000378:metabolism; D006367:HeLa Cells; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D008969:Molecular Sequence Data; D009124:Muscle Proteins / Q000235:genetics / Q000378:metabolism*; D018482:Muscle, Skeletal / Q000378:metabolism*; D010802:Phylogeny; D017386:Sequence Homology, Amino Acid; D015027:Zebrafish / Q000235:genetics* / Q000378:metabolism*; D029961:Zebrafish Proteins
chemical_list: D000911:Antibodies, Monoclonal; D016189:Dystrophin; D008565:Membrane Proteins; D009124:Muscle Proteins; D029961:Zebrafish Proteins; C435224:dmd protein, zebrafish