PUBLICATION
The dystrophin associated protein complex in zebrafish
- Authors
- Guyon, J.R., Mosley, A.N., Zhou, Y., O'Brien, K.F., Sheng, X., Chiang, K., Davidson, A.J., Volinski, J.M., Zon, L.I., and Kunkel, L.M.
- ID
- ZDB-PUB-030307-3
- Date
- 2003
- Source
- Human molecular genetics 12(6): 601-615 (Journal)
- Registered Authors
- Davidson, Alan, Guyon, Jeff, Kunkel, Louis M., Sheng, Xiaoming, Zhou, Yi, Zon, Leonard I.
- Keywords
- none
- MeSH Terms
-
- Amino Acid Sequence
- Animals
- Base Sequence
- Blotting, Western
- Cell Membrane/metabolism
- Chromosome Mapping
- Cloning, Molecular
- Cytoplasm/metabolism
- DNA, Complementary/metabolism
- Databases as Topic
- Dystrophin/biosynthesis*
- Dystrophin/chemistry*
- Electrophoresis, Polyacrylamide Gel
- Exons
- Gene Library
- Humans
- Immunoblotting
- Immunohistochemistry
- Models, Genetic
- Molecular Sequence Data
- Muscle, Skeletal/metabolism
- Muscles/metabolism
- Phenotype
- Polymerase Chain Reaction
- Protein Biosynthesis
- Sequence Homology, Amino Acid
- Zebrafish
- PubMed
- 12620966 Full text @ Hum. Mol. Genet.
Citation
Guyon, J.R., Mosley, A.N., Zhou, Y., O'Brien, K.F., Sheng, X., Chiang, K., Davidson, A.J., Volinski, J.M., Zon, L.I., and Kunkel, L.M. (2003) The dystrophin associated protein complex in zebrafish. Human molecular genetics. 12(6):601-615.
Abstract
Many cases of muscular dystrophy in humans are caused by mutations in members of the dystrophin associated protein complex (DAPC). Zebrafish are small vertebrates whose bodies are composed predominantly of skeletal muscle, making them attractive models for studying mammalian muscle disorders. Potential orthologs to most of the human DAPC proteins have been found in zebrafish by database screening. Expression of the sarcoglycans, dystroglycan and dystrophin has been confirmed by western blotting. Immunohistochemical and biochemical techniques localize these proteins to the muscle cell membrane in adult zebrafish. Morpholino (MO) experiments designed to inhibit the translation of dystrophin mRNA produce juvenile zebrafish that are less active than zebrafish injected with control morpholinos. Western blot analysis of the dystrophin morpholino-injected zebrafish shows concurrent reduction of dystrophin and the sarcoglycans, suggesting that these proteins, like those in mammals, are part of a complex whose integrity is dependant on dystrophin expression. These results indicate that the zebrafish is an excellent animal model in which to approach the study of dystrophin and its associated proteins.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping