CIAO2A Gene

Name cytosolic iron-sulfur assembly component 2A
Description Predicted to enable metal ion binding activity. Involved in iron-sulfur cluster assembly and protein maturation by iron-sulfur cluster transfer. Located in cytosol and nucleoplasm. Part of cytosolic [4Fe-4S] assembly targeting complex. [provided by Alliance of Genome Resources, Mar 2025]
Summary
{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\n Family with sequence similarity 96 member A (CIA2A), also known as FAM96A, is a dedicated component of the cytosolic iron–sulfur (Fe/S) protein assembly (CIA) machinery. Unlike its paralog CIA2B, which partners with CIAO1 and MMS19 to facilitate maturation of most cytosolic–nuclear Fe/S proteins, CIA2A specifically targets iron regulatory protein 1 (IRP1) to ensure its proper incorporation of a [4Fe-4S] cluster. In doing so, the CIA2A–CIAO1 complex catalyzes the conversion of inactive apo-IRP1 to its active aconitase form, which is critical for regulating cellular iron homeostasis; binding of CIA2A is also implicated in the stabilization of IRP2, even though IRP2 lacks an Fe/S cluster."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": " Recent biophysical studies further reveal that CIA2A can form a heterotrimer—with two molecules of CIA2A and one of CIAO1—and bind a [4Fe-4S] cluster via a conserved cysteine (Cys90), thereby providing direct evidence for its role in cluster transfer to IRP1."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "2"}]}, {"type": "t", "text": " Moreover, structural analyses of the DUF59 domain within CIA2A highlight its capability to adopt both monomeric and dimeric, domain‐swapped conformations, which may underlie its functional versatility and tight interaction with other CIA components such as CIAO1."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "4"}]}, {"type": "t", "text": " Collectively, these findings establish CIA2A as a specialized factor in the CIA machinery that secures iron homeostasis by maturing IRP1 and modulating IRP2 levels.\n "}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Oliver Stehling, Judita Mascarenhas, Ajay A Vashisht, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Human CIA2A-FAM96A and CIA2B-FAM96B integrate iron homeostasis and maturation of different subsets of cytosolic-nuclear iron-sulfur proteins."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Cell Metab (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.cmet.2013.06.015"}], "href": "https://doi.org/10.1016/j.cmet.2013.06.015"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23891004"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23891004"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Vincenzo Maione, Francesca Cantini, Mirko Severi, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Investigating the role of the human CIA2A-CIAO1 complex in the maturation of aconitase."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biochim Biophys Acta Gen Subj (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.bbagen.2018.05.019"}], "href": "https://doi.org/10.1016/j.bbagen.2018.05.019"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "29842905"}], "href": "https://pubmed.ncbi.nlm.nih.gov/29842905"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Vincenzo Maione, Deborah Grifagni, Francesco Torricella, et al. "}, {"type": "b", "children": [{"type": "t", "text": "CIAO3 protein forms a stable ternary complex with two key players of the human cytosolic iron-sulfur cluster assembly machinery."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Biol Inorg Chem (2020)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s00775-020-01778-z"}], "href": "https://doi.org/10.1007/s00775-020-01778-z"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "32222833"}], "href": "https://pubmed.ncbi.nlm.nih.gov/32222833"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Caroline Mas, Kai-En Chen, Ian M Brereton, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Backbone resonance assignments of the monomeric DUF59 domain of human Fam96a."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biomol NMR Assign (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1007/s12104-012-9390-1"}], "href": "https://doi.org/10.1007/s12104-012-9390-1"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22618863"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22618863"}]}]}]}
NCBI Gene ID 84191
API
Download Associations
Predicted Functions View CIAO2A's ARCHS4 Predicted Functions.
Co-expressed Genes View CIAO2A's ARCHS4 Predicted Functions.
Expression in Tissues and Cell Lines View CIAO2A's ARCHS4 Predicted Functions.

Functional Associations

CIAO2A has 1,555 functional associations with biological entities spanning 6 categories (functional term, phrase or reference, chemical, disease, phenotype or trait, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 31 datasets.

Click the + buttons to view associations for CIAO2A from the datasets below.

If available, associations are ranked by standardized value

Dataset Summary
Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles tissue samples with high or low expression of CIAO2A gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset.
CCLE Cell Line Proteomics Cell lines associated with CIAO2A protein from the CCLE Cell Line Proteomics dataset.
CellMarker Gene-Cell Type Associations cell types associated with CIAO2A gene from the CellMarker Gene-Cell Type Associations dataset.
ChEA Transcription Factor Targets 2022 transcription factors binding the promoter of CIAO2A gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
CM4AI KOLF21J CRISPRi Gene Perturbation Atlas gene perturbations changing expression of CIAO2A gene from the CM4AI KOLF21J CRISPRi Gene Perturbation Atlas dataset.
COMPARTMENTS Curated Protein Localization Evidence Scores 2025 cellular components containing CIAO2A protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 cellular components co-occuring with CIAO2A protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
DeepCoverMOA Drug Mechanisms of Action small molecule perturbations with high or low expression of CIAO2A protein relative to other small molecule perturbations from the DeepCoverMOA Drug Mechanisms of Action dataset.
DepMap CRISPR Gene Dependency cell lines with fitness changed by CIAO2A gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
DISEASES Text-mining Gene-Disease Association Evidence Scores 2025 diseases co-occuring with CIAO2A gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.
DisGeNET Gene-Disease Associations diseases associated with CIAO2A gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
DisGeNET Gene-Phenotype Associations phenotypes associated with CIAO2A gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset.
GO Biological Process Annotations 2023 biological processes involving CIAO2A gene from the curated GO Biological Process Annotations 2023 dataset.
GO Biological Process Annotations 2025 biological processes involving CIAO2A gene from the curated GO Biological Process Annotations2025 dataset.
GTEx eQTL 2025 SNPs regulating expression of CIAO2A gene from the GTEx eQTL 2025 dataset.
GTEx Tissue Gene Expression Profiles 2023 tissues with high or low expression of CIAO2A gene relative to other tissues from the GTEx Tissue Gene Expression Profiles 2023 dataset.
JASPAR Predicted Human Transcription Factor Targets 2025 transcription factors regulating expression of CIAO2A gene predicted using known transcription factor binding site motifs from the JASPAR Predicted Human Transcription Factor Targets dataset.
JASPAR Predicted Mouse Transcription Factor Targets 2025 transcription factors regulating expression of CIAO2A gene predicted using known transcription factor binding site motifs from the JASPAR Predicted Mouse Transcription Factor Targets 2025 dataset.
NIBR DRUG-seq U2OS MoA Box Gene Expression Profiles drug perturbations changing expression of CIAO2A gene from the NIBR DRUG-seq U2OS MoA Box dataset.
PFOCR Pathway Figure Associations 2023 pathways involving CIAO2A protein from the PFOCR Pathway Figure Associations 2023 dataset.
Replogle et al., Cell, 2022 K562 Essential Perturb-seq Gene Perturbation Signatures gene perturbations changing expression of CIAO2A gene from the Replogle et al., Cell, 2022 K562 Essential Perturb-seq Gene Perturbation Signatures dataset.
Replogle et al., Cell, 2022 K562 Genome-wide Perturb-seq Gene Perturbation Signatures gene perturbations changing expression of CIAO2A gene from the Replogle et al., Cell, 2022 K562 Genome-wide Perturb-seq Gene Perturbation Signatures dataset.
Replogle et al., Cell, 2022 RPE1 Essential Perturb-seq Gene Perturbation Signatures gene perturbations changing expression of CIAO2A gene from the Replogle et al., Cell, 2022 RPE1 Essential Perturb-seq Gene Perturbation Signatures dataset.
Rummagene Transcription Factor Associations 2026 transcription factors regulating expression of CIAO2A gene from the Rummagene Transcription Factor Associations 2026 dataset.
RummaGEO Drug Perturbation Signatures drug perturbations changing expression of CIAO2A gene from the RummaGEO Drug Perturbation Signatures dataset.
RummaGEO Gene Perturbation Signatures gene perturbations changing expression of CIAO2A gene from the RummaGEO Gene Perturbation Signatures dataset.
Sci-Plex Drug Perturbation Signatures drug perturbations changing expression of CIAO2A gene from the Sci-Plex Drug Perturbation Signatures dataset.
TISSUES Curated Tissue Protein Expression Evidence Scores 2025 tissues with high expression of CIAO2A protein from the TISSUES Curated Tissue Protein Expression Evidence Scores 2025 dataset.
TISSUES Experimental Tissue Protein Expression Evidence Scores 2025 tissues with high expression of CIAO2A protein in proteomics datasets from the TISSUES Experimental Tissue Protein Expression Evidence Scores 2025 dataset.
TISSUES Text-mining Tissue Protein Expression Evidence Scores 2025 tissues co-occuring with CIAO2A protein in abstracts of biomedical publications from the TISSUES Text-mining Tissue Protein Expression Evidence Scores 2025 dataset.
WikiPathways Pathways 2024 pathways involving CIAO2A protein from the WikiPathways Pathways 2024 dataset.