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)
Aconitase (ACO2) is an important enzyme located in the mitochondria and catalyzes the interconversion of citrate and isocitrate in the tricarboxylic acid cycle. The expression of this gene may be altered in certain types of cancer. It may play a crutial role in gastric cancer and may potentially serve as a prognostic biomarker. Defect in mitochondrial aconitase is found to be associated with an infantile neurodegenerative disorder affecting mainly the cerebellum and retina.
