SOD1 and Melnick-Needles syndrome: The prevalence of studies documenting alterations in mitochondrial respiration in SOD1 transgenic animal models in contrast to the absence of impairment in mitochondrial respiration observed in studies involving SOD1A4V or SOD1G93A human MNs [66] suggests that mitochondrial dysfunction observed in the SOD1 transgenic animal model may be overestimated and should be carefully interpreted.