Aconitase 2 (ACO2)

Aconitase 2(ACO2) belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitatAconitase 2(ACO2)e 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. Frataxin interacted with aconitase in a citrate-dependent fashion, reduced the level of oxidant-induced inactivation, and converted the inactive [3Fe-4S]1+ enzyme to the active [4Fe-4S]2+ form of the protein.