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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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: Aconitase; aconitase 2, mitochondrial; Aconitate hydratase, mitochondrial; Citrate hydro-lyase; epididymis secretory sperm binding protein Li 284; MGC20605; MGC33908; mitochondrial aconitase; mitochondrial aconitase (nuclear aco2 gene); RP3-347H13.8
Gene Aliases: Aco-2; ACO2; Aco3; ACONM; D10Wsu183e; HEL-S-284; ICRD; OCA8; OPA9
UniProt ID: (Human) Q99798, (Rat) Q9ER34, (Mouse) Q99KI0
Entrez Gene ID: (Human) 50, (Rat) 79250, (Mouse) 11429
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