PUBLICATION
delta-Sarcoglycan is required for early zebrafish muscle organization
- Authors
- 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.
- ID
- ZDB-PUB-050215-3
- Date
- 2005
- 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
- MeSH Terms
-
- Amino Acid Sequence
- Animals
- Base Sequence
- Cloning, Molecular
- Down-Regulation
- Dystrophin/metabolism
- Humans
- Mice
- Molecular Sequence Data
- Muscle, Skeletal/cytology
- Muscle, Skeletal/embryology*
- Sarcoglycans/analysis
- Sarcoglycans/genetics*
- Sarcoglycans/physiology*
- Sarcolemma/chemistry
- Zebrafish/embryology*
- Zebrafish Proteins/genetics*
- Zebrafish Proteins/physiology*
- PubMed
- 15707578 Full text @ Exp. Cell Res.
Citation
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.
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.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping