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Delta-sarcoglycan is required for early zebrafish muscle organization
Authors:Guyon Jeffrey R  Mosley Alycia N  Jun Susan J  Montanaro Federica  Steffen Leta S  Zhou Yi  Nigro Vincenzo  Zon Len I  Kunkel Louis M
Institution:Division of Genetics and Hematology/Oncology, Children's Hospital Boston, USA.
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.
Keywords:Muscular dystrophy  Limb girdle muscular dystrophy  Sarcoglycan  Zebrafish  Sarcolemmal membrane  Myosepta  DAPC
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