Although not pathognomonic, the increased plasma levels of three TCA cycle metabolites (citrate, aconitate, and isocitrate) along with a higher plasma lactate (1.5-fold of controls; p = 0.095) have been observed in some mitochondrial diseases (Crippa et al., 2015), usually associated with the formation of analogs of TCA intermediates with the potential of depleting oxaloacetate and slowing down the TCA cycle, especially if not accompanied by the activation of the anaplerotic reaction catalyzed by pyruvate carboxylase. Here, PC is linked to inborn mitochondrial metabolism disorder.