PUBLICATION
Zebrafish cysteine and glycine-rich protein 3 is essential for mechanical stability in skeletal muscles
- Authors
- Chang, Y., Geng, F., Hu, Y., Ding, Y., Zhang, R.
- ID
- ZDB-PUB-190304-1
- Date
- 2019
- Source
- Biochemical and Biophysical Research Communications 511(3): 604-611 (Journal)
- Registered Authors
- Zhang, Ruilin
- Keywords
- Skeletal muscle, Stretch sensing, Zebrafish, csrp3/mlp
- MeSH Terms
-
- Animals
- Biomechanical Phenomena
- CRISPR-Cas Systems
- Gene Knockdown Techniques
- LIM Domain Proteins/genetics
- LIM Domain Proteins/metabolism*
- Muscle Proteins/genetics
- Muscle Proteins/metabolism*
- Muscle, Skeletal/growth & development
- Muscle, Skeletal/metabolism*
- Muscle, Skeletal/ultrastructure
- Mutation
- Stress, Mechanical
- Zebrafish/genetics
- Zebrafish/growth & development
- Zebrafish/metabolism*
- Zebrafish Proteins/genetics
- Zebrafish Proteins/metabolism*
- PubMed
- 30826063 Full text @ Biochem. Biophys. Res. Commun.
Citation
Chang, Y., Geng, F., Hu, Y., Ding, Y., Zhang, R. (2019) Zebrafish cysteine and glycine-rich protein 3 is essential for mechanical stability in skeletal muscles. Biochemical and Biophysical Research Communications. 511(3):604-611.
Abstract
Cysteine and glycine-rich protein 3 (CSRP3) is a striated muscle-specific cytoskeleton protein which participates in cardiac stretch sensing. Mutations in CSRP3 gene cause cardiomyopathies and deregulation of CSRP3 has been found in patients with heart failure and several skeletal muscle diseases. However, the mechanism underneath these disorders still remains poorly understood. Here we generated the first csrp3 knockout zebrafish. csrp3-/- embryos showed no gross morphological defects but csrp3 deficient skeletal muscle fibers were prone to lesions upon prolonged stretching force. Further studies revealed csrp3 cooperatively interacted with ilk to maintain skeletal muscle mechanical stability and regulated tcap activation. Thus, our work has established a zebrafish model to investigate the function of csrp3 gene, and provides novel insights towards how csrp3 defects may lead to skeletal myopathies by a mechanistic link between Csrp3 and force stimuli.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping