Aliases for ACO2 Gene
External Ids for ACO2 Gene
Previous GeneCards Identifiers for ACO2 Gene
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]
GeneCards Summary for ACO2 Gene
ACO2 (Aconitase 2, Mitochondrial) is a Protein Coding gene. Diseases associated with ACO2 include infantile cerebellar-retinal degeneration and athetosis. Among its related pathways are Metabolism and Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein. GO annotations related to this gene include iron ion binding and aconitate hydratase activity.
UniProtKB/Swiss-Prot for ACO2 Gene
Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.