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CISD3 is required for Complex I function, mitochondrial integrity, and skeletal muscle maintenance.
Biorxiv : the Preprint Server for Biology
|July 3, 2023
Summary
Mitochondrial protein CISD3 (CDG-SH3 domain-containing protein 3) is crucial for muscle maintenance. Its deficiency causes muscle atrophy and impairs mitochondrial function, highlighting its potential therapeutic role in aging and muscle diseases.
Area of Science:
- Mitochondrial biology
- Skeletal muscle physiology
- Protein biochemistry
Background:
- Mitochondria are vital for muscle metabolism and function.
- CISD proteins are iron-sulfur proteins supporting mitochondrial health.
- Reduced CISD protein levels are linked to age-related muscle degeneration.
Approach:
- Investigated the role of the inner mitochondrial protein CISD3 in mouse models.
- Analyzed proteomic and functional changes in skeletal muscle mitochondria.
- Examined the interaction between CISD3 and Complex I respiratory chain subunit NDUFV2.
Key Points:
- CISD3 deficiency in mice leads to muscle atrophy with proteomic similarities to Duchenne Muscular Dystrophy.
- Mice lacking CISD3 exhibit impaired skeletal muscle mitochondrial function and structure.
- CISD3 interacts with and donates iron-sulfur clusters to NDUFV2, a subunit of Complex I.
Conclusions:
- CISD3 is essential for the biogenesis and function of mitochondrial Complex I.
- This finding underscores CISD3's importance in maintaining muscle function and integrity.
- Targeting CISD3 may offer therapeutic strategies for muscle degeneration and aging.
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