Journal
EXPERIMENTAL CELL RESEARCH
Volume 304, Issue 1, Pages 105-115Publisher
ELSEVIER INC
DOI: 10.1016/j.yexcr.2004.10.032
Keywords
muscular dystrophy; limb girdle muscular dystrophy; Sarcoglycan; zebrafish; sarcolemmal membrane; myosepta; DAPC
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Funding
- NIDDK NIH HHS [1R01 DK55381, 5P50 DK49216] Funding Source: Medline
- Telethon [TGM06S01, TGM03Z02] Funding Source: Medline
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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. (C) 2004 Elsevier Inc. All rights reserved.
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