Mutations in the human VAPB (hVAPB) is an important risk factor for ALS and deficiency of VAPB causes LDs accumulation and clustering in muscles, which may constitute a compensatory mechanism counterbalancing skeletal muscle mitochondrial dysfunction in C. elegans, since the TAG accumulation in muscle helps to maintain the ATP levels via β-oxidation [185]. Here, VAPB is linked to amyotrophic lateral sclerosis.