ACO1

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	MGI: 87879 HomoloGene: 1657 GeneCards: ACO1
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 • metabolic process • 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_007386
RefSeq (protein)	NP_001265281 NP_002188	NP_031412
Location (UCSC)	Chr 9: 32.38 – 32.45 Mb	Chr 4: 40.14 – 40.2 Mb
PubMed search

2B3X, 2B3Y

48

11428

ENSG00000122729

ENSMUSG00000028405

P21399

P28271

NM_001278352
NM_002197

NM_007386

NP_001265281
NP_002188

NP_031412

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

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.

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