ACO1 and hereditary myopathy with lactic acidosis due to ISCU deficiency: The P144L mutation also causes iron deposition in the cells, similarly to what has been reported in ISCU myopathy (Kollberg et al. 2009; Olsson et al. 2008), in agreement with the role of FDX2 in cellular iron homeostasis via its mitochondrial role in the maturation of cytosolic IRP1 to an aconitase.