PER1 is required for GPI-phospholipase A2 activity and is involved in lipid remodelling of GPI-anchored proteins []. PER1 is part of the CREST superfamily []. Human PERLD1 is a functional homologue of PER1, and is also known as PGAP3. Mutations in PGAP3 cause hyperphosphatasia with mental retardation syndrome [].
PGAP2, also known as FRAG1, is involved in the lipid remodeling steps of glycosylphosphatidylinositol (GPI)-anchor maturation []. Immediately after attachment of GPI to newly synthesized proteins, inositol-linked palmitate is removed by deacylase PGAP1 in the ER. The inositol-deacylation is essential for subsequent steps in fatty acid remodeling in the Golgi. PGAP3 removes an unsaturated fatty chain at sn-2 and PGAP2 reacylation of sn-2 with stearic acid follows [].
