ACO1

Aconitase 1, soluble is a protein that in humans is encoded by the ACO1 gene.^[5]

ACO1
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2B3X, 2B3Y
Identifiers
Aliases	ACO1, ACONS, HEL60, IREB1, IREBP, IREBP1, IRP1, aconitase 1
External IDs	OMIM: 100880; MGI: 87879; HomoloGene: 1657; GeneCards: ACO1; OMA:ACO1 - orthologs
Gene location (Human) Chr. Chromosome 9 (human)[1] Band 9p21.1 Start 32,384,603 bp[1] End 32,454,769 bp[1]
Gene location (Mouse) Chr. Chromosome 4 (mouse)[2] Band 4 A5|4 20.24 cM Start 40,143,081 bp[2] End 40,198,338 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in right lobe of liver stromal cell of endometrium right adrenal gland right adrenal cortex adipose tissue left adrenal gland kidney tubule subcutaneous adipose tissue duodenum jejunal mucosa Top expressed in tunica adventitia of aorta external carotid artery internal carotid artery migratory enteric neural crest cell dermis efferent ductule carotid body tunica media of zone of aorta submandibular gland proximal tubule More reference expression data BioGPS n/a
Gene ontology Molecular function iron-sulfur cluster binding metal ion binding protein binding lyase activity RNA binding aconitate hydratase activity iron-responsive element binding 3 iron, 4 sulfur cluster binding 4 iron, 4 sulfur cluster binding Cellular component Golgi apparatus endoplasmic reticulum mitochondrion extracellular exosome cytoplasm cytosol Biological process response to iron(II) ion tricarboxylic acid cycle post-embryonic development regulation of gene expression intestinal absorption metabolism regulation of translation citrate metabolic process cellular iron ion homeostasis Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 48 11428 Ensembl ENSG00000122729 ENSMUSG00000028405 UniProt P21399 P28271 RefSeq (mRNA) NM_001278352 NM_002197 NM_001362840 NM_007386 RefSeq (protein) NP_001265281 NP_002188 NP_001349769 NP_031412 Location (UCSC) Chr 9: 32.38 – 32.45 Mb Chr 4: 40.14 – 40.2 Mb PubMed search [3] [4]
Wikidata
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Function

The protein encoded by this gene is a bifunctional, cytosolic protein that functions as an essential enzyme in the TCA cycle and interacts with mRNA to control the levels of iron inside cells. When cellular iron levels are high, this protein binds to a 4Fe-4S cluster and functions as an aconitase. Aconitases are iron-sulfur proteins that function to catalyze the conversion of citrate to isocitrate. When cellular iron levels are low, the protein binds to iron-responsive elements (IREs), which are stem-loop structures found in the 5' UTR of ferritin mRNA, and in the 3' UTR of transferrin receptor mRNA. When the protein binds to IRE, it results in repression of translation of ferritin mRNA, and inhibition of degradation of the otherwise rapidly degraded transferrin receptor mRNA. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Alternative splicing results in multiple transcript variants.^[5]