Of note, in its non-IRE binding form IRP-1 acts as a cytoplasmic aconitase with currently unknown physiological function whereas the translation of mitochondrial aconitase is controlled by IRE/IRP interaction in a way that surplus iron promotes mitochondrial aconitase expression whereas iron deficiency results in repression of mitochondrial aconitase translation [7, 8]. The gene discussed is ACO2; the disease is nutritional disorder.