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ACO1

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Identifiers
SymbolsACO1 ; ACONS; HEL60; IREB1; IREBP; IREBP1; IRP1
External IDsOMIM100880 HomoloGene1657 GeneCards: ACO1 Gene
EC number4.2.1.3
Orthologs
SpeciesHumanMouse
Entrez4811428
EnsemblENSG00000122729ENSMUSG00000028405
UniProtP21399P28271
RefSeq (mRNA)NM_001278352NM_007386
RefSeq (protein)NP_001265281NP_031412
Location (UCSC)Chr 9:
32.38 – 32.45 Mb
Chr 4:
40.14 – 40.2 Mb
PubMed search[1][2]

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

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.[1]

References

Further Reading

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