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.
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Protein Aliases: Aconitase; aconitase 2, mitochondrial; Aconitate hydratase, mitochondrial; Citrate hydro-lyase; epididymis secretory sperm binding protein Li 284; MGC20605; MGC33908; mitochondrial aconitase; RP3-347H13.8
Gene Aliases: ACO2; ACONM; HEL-S-284; ICRD; OCA8; OPA9
UniProt ID: (Human) Q99798
Entrez Gene ID: (Human) 50