PUBLICATION
Modeling human muscle disease in zebrafish
- Authors
- Guyon, J.R., Steffen, L.S., Howell, M.H., Pusack, T.J., Lawrence, C., and Kunkel, L.M.
- ID
- ZDB-PUB-060906-6
- Date
- 2007
- Source
- Biochimica et biophysica acta. Molecular basis of disease 1772(2): 205-215 (Review)
- Registered Authors
- Guyon, Jeff, Kunkel, Louis M., Lawrence, Christian, Steffen, Leta Suzanne
- Keywords
- Zebrafish, Muscular dystrophy, Dystrophin, Animal models, Therapy
- MeSH Terms
-
- Animals
- Disease Models, Animal*
- Humans
- Muscular Dystrophies/etiology
- Muscular Dystrophies/genetics*
- Muscular Dystrophies/pathology*
- Muscular Dystrophies/therapy
- Zebrafish/genetics*
- Zebrafish/metabolism
- PubMed
- 16934958 Full text @ BBA Molecular Basis of Disease
Citation
Guyon, J.R., Steffen, L.S., Howell, M.H., Pusack, T.J., Lawrence, C., and Kunkel, L.M. (2007) Modeling human muscle disease in zebrafish. Biochimica et biophysica acta. Molecular basis of disease. 1772(2):205-215.
Abstract
Zebrafish reproduce in large quantities, grow rapidly, and are transparent early in development. For these reasons, zebrafish have been used extensively to model vertebrate development and disease. Like mammals, zebrafish express dystrophin and many of its associated proteins early in development and these proteins have been shown to be vital for zebrafish muscle stability. In dystrophin-null zebrafish, muscle degeneration becomes apparent as early as 3 days post-fertilization (dpf) making the zebrafish an excellent organism for large-scale screens to identify other genes involved in the disease process or drugs capable of correcting the disease phenotype. Being transparent, developing zebrafish are also an ideal experimental model for monitoring the fate of labeled transplanted cells. Although zebrafish dystrophy models are not meant to replace existing mammalian models of disease, experiments requiring large numbers of animals may be best performed in zebrafish. Results garnered from using this model could lead to a better understanding of the pathogenesis of the muscular dystrophies and the development of future therapies.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping