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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CIAO2B
cytosolic iron-sulfur assembly component 2B
Chromosome 16 · 16q22.1
NCBI Gene: 51647Ensembl: ENSG00000166595.12HGNC: HGNC:24261UniProt: Q9Y3D0
77PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingchromosome segregationcytosolMMXD complexhepatocellular carcinomaneoplasmidiopathic hypereosinophilic syndromehereditary neutrophilia
✦AI Summary

CIAO2B (cytosolic iron-sulfur assembly component 2B) is a central component of the cytosolic iron-sulfur (Fe-S) cluster assembly (CIA) pathway. As part of the CIA targeting complex, CIAO2B bridges CIAO1 and the client adaptor MMS19 to facilitate Fe-S cluster incorporation into cytosolic and nuclear proteins 1. The CIA2B-CIA1-MMS19 complex assembles most cytosolic-nuclear Fe-S proteins involved in DNA replication, repair, protein translation, and iron homeostasis 2. CIAO2B also functions within the mitotic spindle-associated MMXD complex, facilitating Fe-S cluster assembly into ERCC2/XPD to promote proper chromosome 16 3. Additionally, CIAO2B (FAM96B) transfers Fe-S clusters to the motor protein KIF4A, ensuring its correct mitotic spindle localization 4. Beyond Fe-S cluster assembly, CIAO2B recruits creatine kinase to the mitotic spindle, coordinating ATP and Fe-S cluster supply during mitosis 5. CIAO2B interactions with selenoprotein W and prelamin A suggest broader roles in brain development and cellular senescence 67. CIAO2B depletion causes mitotic defects, aberrant chromosome 16, and impaired stem cell function, highlighting its clinical relevance to chr16 instability and cellular regeneration 8.

Sources cited
1
CIAO2B is centrally located in the CIA targeting complex, bridging CIAO1 and MMS19, with structural details of client protein recognition
PMID: 32632277
2
CIA2B-CIA1-MMS19 complex binds to and facilitates assembly of most cytosolic-nuclear Fe-S proteins
PMID: 23891004
3
FAM96B is part of the MMXD complex required for chromosome segregation via XPD/ERCC2 Fe-S cluster assembly
PMID: 20797633
4
MIP18 (FAM96B) interacts with CIAO1 and Fe-S proteins in the CIA machinery
PMID: 23150669
5
FAM96B recruits brain-type creatine kinase to the mitotic spindle to coordinate ATP and Fe-S cluster homeostasis
PMID: 36503010
6
FAM96B is a novel binding partner of selenoprotein W in the brain
PMID: 30876693
7
FAM96B is a prelamin A binding partner with potential involvement in cellular senescence
PMID: 24041693
8
FAM96B suppresses dental pulp stem cell senescence and promotes osteogenic differentiation
PMID: 32039527
Disease Associationsⓘ20
hepatocellular carcinomaOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.05Suggestive
idiopathic hypereosinophilic syndromeOpen Targets
0.04Suggestive
hereditary neutrophiliaOpen Targets
0.04Suggestive
Felty's syndromeOpen Targets
0.04Suggestive
dominant beta-thalassemiaOpen Targets
0.04Suggestive
hemoglobin H diseaseOpen Targets
0.04Suggestive
acute erythroleukemiaOpen Targets
0.04Suggestive
erythroleukemia, familial, susceptibility toOpen Targets
0.04Suggestive
Hemoglobin C - beta-thalassemiaOpen Targets
0.04Suggestive
hemoglobin C-beta-thalassemia syndromeOpen Targets
0.04Suggestive
glycoprotein storage diseaseOpen Targets
0.04Suggestive
combined immunodeficiency due to OX40 deficiencyOpen Targets
0.04Suggestive
hemolytic anemia due to diphosphoglycerate mutase deficiencyOpen Targets
0.04Suggestive
chronic myelogenous leukemiaOpen Targets
0.04Suggestive
immunodeficiency 84Open Targets
0.03Suggestive
breast cancerOpen Targets
0.03Suggestive
Hyperlipoproteinemia type 1Open Targets
0.03Suggestive
Hereditary persistence of fetal hemoglobin - beta-thalassemiaOpen Targets
0.03Suggestive
hereditary persistence of fetal hemoglobin-beta-thalassemia syndromeOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SLC25A5Protein interaction100%SLC25A6Protein interaction100%ERCC2Protein interaction100%NUBP1Protein interaction99%CIAO3Protein interaction99%CIAPIN1Protein interaction90%
Tissue Expression6 tissues
Liver
100%
Lung
82%
Heart
81%
Brain
76%
Ovary
73%
Bone Marrow
37%
Gene Interaction Network
Click a node to explore
CIAO2BSLC25A5SLC25A6ERCC2NUBP1CIAO3CIAPIN1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9Y3D0
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.68LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.17 [0.82–1.68]
RankingsWhere CIAO2B stands among ~20K protein-coding genes
  • #6,142of 20,598
    Most Researched77
  • #16,026of 17,882
    Most Constrained (LOEUF)1.68
Genes detectedCIAO2B
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Structural insights into Fe-S protein biogenesis by the CIA targeting complex.
PMID: 32632277
Nat Struct Mol Biol · 2020
1.00
2
Human CIA2A-FAM96A and CIA2B-FAM96B integrate iron homeostasis and maturation of different subsets of cytosolic-nuclear iron-sulfur proteins.
PMID: 23891004
Cell Metab · 2013
0.90
3
Fam96b recruits brain-type creatine kinase to fuel mitotic spindle formation.
PMID: 36503010
Biochim Biophys Acta Mol Cell Res · 2023
0.80
4
FAM96B inhibits the senescence of dental pulp stem cells.
PMID: 32039527
Cell Biol Int · 2020
0.70
5
Identification of FAM96B as a novel selenoprotein W binding partner in the brain.
PMID: 30876693
Biochem Biophys Res Commun · 2019
0.60