Finally, the significance of this complex is highlighted in humans where loss-of-function mutations in FIG4 and VAC14 genes result in severe neurological disorders including Amyotrophic Lateral Sclerosis (ALS), Charcot Marie Tooth Type 4 J and Yunis-Varón Syndrome13,15–18. This evidence concerns the gene VAC14 and amyotrophic lateral sclerosis.