ACO2 antibody (FNab00088)

ACO2(aconitate hydratase, mitochondrial) is also named as citrate hydro-lyase and belongs to the aconitase/IPM isomerase family. It plays a key function in cellular energy production, and loss of its activity has a major impact on cellular and organismal survival. Western blot shows two bands of 83 kDa and 40 kDa. The 40 kDa fragment decreases with age and oxidative stress, whereas other fragmentation products with molecular weights between 40 and 83 kDa increased with age and MnSOD(mitochondrial manganese superoxide dismutase) deficiency(PMID:12459471). Defects in ACO2 are the cause of infantile cerebellar-retinal degeneration(ICRD).