Moreover, it has been shown that the phospholipase PNPLA1 belonging to the iPLA2 family in murine keratinocytes plays a crucial role in the biosynthesis of ω-O-acylceramide, a lipid component essential for the functioning of the skin barrier [61], and mutations in the PNPLA1 enzyme in both C- and N-terminal domains are associated with the development of autosomal recessive congenital ichthyosis (ARCI) [69, 70] causing abnormal lipid accumulation in fibroblasts and impairing the biosynthesis of ω-O-acylceramide [62]. Here, PNPLA1 is linked to autosomal recessive congenital ichthyosis.