ACO2, Official Symbol ACO2provided by HGNC Official Full Name aconitase 2provided by HGNC Primary source HGNC:HGNC:118 See related Ensembl:ENSG00000100412 MIM:100850; AllianceGenome:HGNC:118 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as ICRD; OCA8; OPA9; ACONM; HEL-S-284 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] Expression Broad expression in heart (RPKM 162.7), kidney (RPKM 93.6) and 24 other tissues See more Orthologs mouse all