Main pageKEGG pathway → Glycosylphosphatidylinositol(GPI)-anchor biosynthesis

KEGG pathway: Glycosylphosphatidylinositol(GPI)-anchor biosynthesis

Pathway hsa00563
Name Glycosylphosphatidylinositol(GPI)-anchor biosynthesis
Members PIGW PIGN PIGC PIGT PIGM PIGA DPM2 PIGF PIGY PIGG PIGU PIGO PIGL PIGS PIGB PIGK GPAA1 PIGV PGAP1 PIGP PIGH PIGX PIGQ PIGZ GPLD1
Description Cell surface proteins can be attached to the cell membrane via the glycolipid structure called glycosylphosphatidylinositol (GPI) anchor. Hundreds of GPI-anchored proteins have been identified in many eukaryotes ranging from protozoa and fungi to mammals. All protein-linked GPI anchors share a common core structure, characterized by the substructure Man (a1-4) GlcN (a1-6) myo-inositol-1P-lipid. Biosynthesis of GPI anchors proceeds in three stages: (i) preassembly of a GPI precursor in the ER membrane, (ii) attachment of the GPI to the C-terminus of a newly synthesized protein in the lumen of the ER, and (iii) lipid remodeling and/or carbohydrate side-chain modifications in the ER and the Golgi. Defects of GPI anchor biosynthesis gene result in a genetic disorder, paroxysmal nocturnal hemoglobinuria.