aconitase 1, soluble


ACO1 (may also be known as: None)




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 [provided by RefSeq, Jan 2014]


aco-1 Caenorhabditis elegans
Irp-1A Drosophila melanogaster
aco1 Danio rerio
Aco1 Mus musculus
Aco1 Rattus norvegicus

Links to external resources

Changes associated with this gene

GO Terms

GO IDGO TermGO Category
GO:0006099 tricarboxylic acid cycle biological_process
GO:0006101 citrate metabolic process biological_process
GO:0006417 regulation of translation biological_process
GO:0006879 cellular iron ion homeostasis biological_process
GO:0008152 metabolic process biological_process
GO:0009791 post-embryonic development biological_process
GO:0010040 response to iron(II) ion biological_process
GO:0010468 regulation of gene expression biological_process
GO:0050892 intestinal absorption biological_process
GO:0005625 soluble fraction cellular_component
GO:0005737 cytoplasm cellular_component
GO:0005739 mitochondrion cellular_component
GO:0005783 endoplasmic reticulum cellular_component
GO:0005792 microsome cellular_component
GO:0005794 Golgi apparatus cellular_component
GO:0005829 cytosol cellular_component
GO:0003723 RNA binding molecular_function
GO:0003994 aconitate hydratase activity molecular_function
GO:0005515 protein binding molecular_function
GO:0016829 lyase activity molecular_function
GO:0030350 iron-responsive element binding molecular_function
GO:0046872 metal ion binding molecular_function
GO:0048027 mRNA 5'-UTR binding molecular_function
GO:0051539 4 iron, 4 sulfur cluster binding molecular_function
GO:0052632 citrate hydro-lyase (cis-aconitate-forming) activity molecular_function
GO:0052633 isocitrate hydro-lyase (cis-aconitate-forming) activity molecular_function