Ether lipids (EL) are glycerophospholipids with a different structure and functional characteristics than fatty acids, thus requiring a specialized biosynthesis pathway. Peroxisomal fatty acyl-CoA reductase 1 (FAR1) is a rate-limiting enzyme for ether lipid synthesis, with mutations associated with a rare human disease. Moreover, altered EL homeostasis has been observed in various prevalent human diseases.