Human Aconitate Hydratase, Mitochondrial (ACO2) Protein

Background

Aconitase 2 (ACO2) is a mitochondrial enzyme involved in the tricarboxylic acid (TCA) cycle, where it catalyzes the reversible isomerization of citrate to isocitrate via cis-aconitate. ACO2 is essential for cellular energy production, linking carbohydrate metabolism to the production of reducing equivalents such as NADH and FADH2. The enzyme contains an iron-sulfur cluster that is critical for its catalytic activity but also makes it sensitive to oxidative damage, which can impair function. Mutations or dysregulation of ACO2 are associated with mitochondrial diseases, neurodegenerative disorders such as Parkinson's disease, and aging, as oxidative stress often leads to loss of aconitase activity. ACO2 is also being explored as a biomarker for mitochondrial dysfunction in metabolic and neurological conditions.