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Scheme of the physiological and defective citric acid cycle in aconitase 2 deficiency with an overview of the CAC with its intermediates and bypassing Triheptanoin. The position of the aconitase 2 enzyme in CAC is illustrated as ACO2. Blocked pathway by defective ACO2 marked with a red line. Triheptanoin bypasses aconitase 2 and feeds the CAC as succinyl-CoA.
