CIAO1 encodes cytosolic iron-sulfur assembly component 1 (339 aa). Key component of the cytosolic iron-sulfur protein assembly (CIA) complex, a multiprotein complex that mediates the incorporation of iron-sulfur cluster into extramitochondrial Fe/S proteins. Highest expression in Cells Cultured fibroblasts (79.0 TPM) and Cells EBV-transformed lymphocytes (76.2 TPM).

Multiple mitochondrial dysfunctions syndrome 10 is associated with mutations in the CIAO1 gene on chromosome 2.

The CIAO1 protein participates in Cytosolic iron-sulfur cluster assembly pathway.

CIAO1 is classified as a druggable target with score 0.0.

Genetic testing for CIAO1 is available. Testing is considered confirmatory for diagnosis.

No clinical trials have been registered for multiple mitochondrial dysfunctions syndrome 10.

6 publications have been identified in PubMed for multiple mitochondrial dysfunctions syndrome 10. Research spans Basic Science / Preclinical (67%), Case Report / Case Series (17%), and Epidemiology / Natural History (17%).

Recent Publications

• Hu H (2026). [PMID: 41319277](https://pubmed.ncbi.nlm.nih.gov/41319277/). *Adv Sci (Weinh)*. [Basic Science / Preclinical]

• Doni D (2025). [PMID: 41372147](https://pubmed.ncbi.nlm.nih.gov/41372147/). *Cell Death Dis*. [Case Report / Case Series]

• Shalash R (2025). [PMID: 40304211](https://pubmed.ncbi.nlm.nih.gov/40304211/). *Aging Cell*. [Basic Science / Preclinical]

• Jiang H (2025). [PMID: 39227420](https://pubmed.ncbi.nlm.nih.gov/39227420/). *J Hum Genet*. [Epidemiology / Natural History]

• Maio N (2024). [PMID: 38950322](https://pubmed.ncbi.nlm.nih.gov/38950322/). *J Clin Invest*. [Basic Science / Preclinical]