|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.
|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|
|PubMed:||15707578 Full text @ Exp. Cell Res.|
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. (2005) delta-Sarcoglycan is required for early zebrafish muscle organization. Experimental cell research. 304(1):105-115.
ABSTRACTMutations 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.