Homo sapiens Gene: ACO2
Summary
InnateDB Gene IDBG-9203.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol ACO2
Gene Name aconitase 2, mitochondrial
Synonyms ACONM; ICRD;
Species Homo sapiens
Ensembl Gene ENSG00000100412
Encoded Proteins
aconitase 2, mitochondrial
aconitase 2, mitochondrial
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification. [provided by RefSeq, Jul 2008]
Gene Information
Type Protein coding
Genomic Location Chromosome 22:41469125-41528989
Strand Forward strand
Band q13.2
Transcripts
ENST00000216254 ENSP00000216254
ENST00000396512 ENSP00000379769
ENST00000471094
ENST00000482208
ENST00000478010
ENST00000481310
ENST00000466237
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 20 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated
Total 20 [view]
Protein-Protein 19 [view]
Protein-DNA 1 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0003994 aconitate hydratase activity
GO:0005506 iron ion binding
GO:0005515 protein binding
GO:0051538 3 iron, 4 sulfur cluster binding
GO:0051539 4 iron, 4 sulfur cluster binding
Biological Process
GO:0006091 generation of precursor metabolites and energy
GO:0006099 tricarboxylic acid cycle
GO:0006101 citrate metabolic process
GO:0006102 isocitrate metabolic process
GO:0008152 metabolic process
GO:0008219 cell death
GO:0044237 cellular metabolic process
GO:0044281 small molecule metabolic process
Cellular Component
GO:0005634 nucleus
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
Method
Confidence
Comments
SSD Ortholog
Ortholog supports species divergence
Not yet available
SSD Ortholog
Ortholog supports species divergence
Pathways
NETPATH
REACTOME
Pyruvate metabolism and Citric Acid (TCA) cycle pathway
Citric acid cycle (TCA cycle) pathway
Metabolism of proteins pathway
The citric acid (TCA) cycle and respiratory electron transport pathway
Metabolism pathway
Mitochondrial protein import pathway
KEGG
Citrate cycle (TCA cycle) pathway
Glyoxylate and dicarboxylate metabolism pathway
INOH
Citrate cycle pathway
PID BIOCARTA
PID NCI
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.643610
RefSeq NM_001098
HUGO
OMIM
CCDS CCDS14017
HPRD 00012
IMGT
EMBL
GenPept
RNA Seq Atlas