Homo sapiens Gene: ACO1
Summary
InnateDB Gene IDBG-55575.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol ACO1
Gene Name aconitase 1, soluble
Synonyms ACONS; HEL60; IREB1; IREBP; IREBP1; IRP1
Species Homo sapiens
Ensembl Gene ENSG00000122729
Encoded Proteins
aconitase 1, soluble
aconitase 1, soluble
aconitase 1, soluble
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
InnateDB Annotation from Orthologs
Summary
[Mus musculus] Macrophage iron regulatory proteins Aco1 and Ireb2 are protective against Salmonella by promoting the induction of Lcn2, a host antimicrobial factor that inhibits bacterial uptake of iron-laden siderophores, and by suppressing the ferritin iron pool.
Entrez Gene
Summary Aconitase 1, also known as iron regulatory element binding protein 1 (IREB1), is a cytosolic protein which binds to iron-responsive elements (IREs). IREs are stem-loop structures found in the 5' UTR of ferritin mRNA, and in the 3' UTR of transferrin receptor mRNA. The iron-induced binding to the IRE results in repression of translation of ferritin mRNA, and inhibition of degradation of the otherwise rapidly degrading transferrin receptor mRNA. Thus, IREB1 plays a central role in cellular iron homeostasis. It was also shown to have aconitase activity, and hence grouped with the aconitase family of enzymes. [provided by RefSeq, Jul 2008]
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]
Gene Information
Type Protein coding
Genomic Location Chromosome 9:32384603-32454769
Strand Forward strand
Band p21.1
Transcripts
ENST00000379923 ENSP00000369255
ENST00000309951 ENSP00000309477
ENST00000541043 ENSP00000438733
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 14 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated
Total 14 [view]
Protein-Protein 12 [view]
Protein-DNA 1 [view]
Protein-RNA 0
DNA-DNA 1 [view]
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0003723 RNA binding
GO:0003994 aconitate hydratase activity
GO:0005515 protein binding
GO:0030350 iron-responsive element binding
GO:0046872 metal ion binding
GO:0051539 4 iron, 4 sulfur cluster binding
Biological Process
GO:0006099 tricarboxylic acid cycle
GO:0006101 citrate metabolic process
GO:0006417 regulation of translation
GO:0006879 cellular iron ion homeostasis
GO:0008152 metabolic process
GO:0009791 post-embryonic development
GO:0010040 response to iron(II) ion
GO:0010468 regulation of gene expression
GO:0050892 intestinal absorption
Cellular Component
GO:0005737 cytoplasm
GO:0005739 mitochondrion
GO:0005783 endoplasmic reticulum
GO:0005794 Golgi apparatus
GO:0005829 cytosol
GO:0070062 extracellular vesicular exosome
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
Not yet available
Pathways
NETPATH
REACTOME
KEGG
Citrate cycle (TCA cycle) pathway
Glyoxylate and dicarboxylate metabolism pathway
INOH
Citrate cycle pathway
PID NCI
Cross-References
SwissProt P21399
TrEMBL Q9HBB2 V9HWB7
UniProt Splice Variant
Entrez Gene 48
UniGene Hs.715195
RefSeq NM_001278352 NM_002197 XM_005251476 XM_006716777
HUGO HGNC:117
OMIM 100880
CCDS CCDS6525
HPRD
IMGT
EMBL AF261088 AL161783 BC018103 CH471071 DQ496106 EU794627 M58510 Z11559
GenPept AAA69900 AAF99681 AAH18103 ABF47095 ACJ13681 CAA77651 CAH72598 EAW58549 EAW58550 EAW58552
RNA Seq Atlas 48