PUBLICATION
Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology
- Authors
- Berger, J., Berger, S., Hall, T.E., Lieschke, G.J., and Currie, P.D.
- ID
- ZDB-PUB-101004-16
- Date
- 2010
- Source
- Neuromuscular disorders : NMD 20(12): 826-832 (Journal)
- Registered Authors
- Berger, Joachim, Berger, Silke, Currie, Peter D., Hall, Thomas, Lieschke, Graham J.
- Keywords
- Dystrophin, DMD, Duchenne muscular dystrophy, Zebrafish, Muscle
- MeSH Terms
-
- Animals
- Animals, Genetically Modified
- Disease Models, Animal
- Dystrophin/genetics*
- Dystrophin/metabolism
- Immunohistochemistry
- Muscle, Skeletal/metabolism
- Muscle, Skeletal/pathology*
- Muscular Dystrophy, Duchenne/genetics*
- Muscular Dystrophy, Duchenne/metabolism
- Muscular Dystrophy, Duchenne/pathology
- Phenotype
- Zebrafish
- PubMed
- 20850317 Full text @ Neuromuscul. Disord.
Citation
Berger, J., Berger, S., Hall, T.E., Lieschke, G.J., and Currie, P.D. (2010) Dystrophin-deficient zebrafish feature aspects of the Duchenne muscular dystrophy pathology. Neuromuscular disorders : NMD. 20(12):826-832.
Abstract
Duchenne muscular dystrophy is caused by mutations in the dystrophin gene. As in humans, zebrafish dystrophin is initially expressed at the peripheral ends of the myofibres adjacent to the myotendinous junction and gradually shifts to non-junctional sites. Dystrophin-deficient zebrafish larvae are characterised by abundant necrotic fibres being replaced by mono-nucleated infiltrates, extensive fibrosis accompanied by inflammation, and a broader variation in muscle fibre cross-sectional areas. Muscle progenitor proliferation cannot compensate for the extensive skeletal muscle loss. Live imaging of dystrophin-deficient zebrafish larvae documents detaching myofibres elicited by muscle contraction. Correspondingly, the progressive phenotype of dystrophin-deficient zebrafish resembles many aspects of the human disease, suggesting that specific advantages of the zebrafish model system, such as the ability to undertake in vivo drug screens and real time analysis of muscle fibre loss, could be used to make novel insights relevant to understanding and treating the pathological basis of dystrophin-deficient muscular dystrophy.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping