LIPA and lysosomal acid lipase deficiency: Its metabolic relevance is underscored by the fact that patients with loss-of-function mutations in the LAL-encoding LIPA gene (3, 4) suffer from LAL deficiency (LAL-D; MIM#278000), a rare autosomal recessive disorder with an estimated prevalence of 1 per 177,000 individuals (3, 4).