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Ccn6 Is Required for Mitochondrial Integrity and Skeletal Muscle Function in Zebrafish
Archya Sengupta1, Deepesh Kumar Padhan1, Ananya Ganguly1
1Division of Cancer Biology & Inflammatory Disorder, CSIR-Indian Institute of Chemical Biology, Kolkata, India.
Frontiers in Cell and Developmental Biology
|March 1, 2021
Summary
CCN6 (WISP3) protein is crucial for mitochondrial function in skeletal muscles. Its depletion impairs mitochondrial complexes, leading to muscle defects, suggesting a role in progressive pseudorheumatoid dysplasia.
Area of Science:
- Molecular Biology
- Genetics
- Zebrafish Models
Background:
- Mutations in the CCN6 (WISP3) gene cause progressive pseudorheumatoid dysplasia (PPRD), a severe musculoskeletal disorder.
- The precise role of CCN6 in musculoskeletal health is not fully understood.
Purpose of the Study:
- To investigate the functional significance of CCN6 in skeletal muscle maintenance.
- To explore the molecular mechanisms underlying CCN6's role in muscle function using a zebrafish model.
Main Methods:
- Utilized zebrafish as a model organism to study Ccn6 function.
- Employed morpholino-mediated depletion to reduce Ccn6 levels in skeletal muscle.
- Analyzed mitochondrial respiratory complex assembly, activity, and abundance.
- Assessed skeletal muscle architecture and function.
Main Results:
- Zebrafish Ccn6 was identified as a component of mitochondrial respiratory complexes in skeletal muscle.
- Ccn6 depletion resulted in reduced mitochondrial respiratory complex assembly and activity.
- Loss of Ccn6 led to decreased mitochondrial abundance and muscle structural/functional deficits.
- Mitochondrial deficiencies correlated with impaired skeletal muscle integrity.
Conclusions:
- CCN6 plays a vital role in regulating mitochondrial respiratory complex assembly, activity, and integrity in skeletal muscle.
- These findings highlight the importance of CCN6 for maintaining skeletal muscle structure and function.
- Defects in CCN6-dependent mitochondrial function may underlie the muscle weakness observed in progressive pseudorheumatoid dysplasia (PPRD).
