Entry Name Glycosylphosphatidylinositol (GPI)-anchor biosynthesis - Homo sapiens (human)
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.
Class Metabolism; Glycan biosynthesis and metabolismBRITE hierarchy
Pathway map hsa00563 Glycosylphosphatidylinositol (GPI)-anchor biosynthesis
Module Network Element N00748 GPI-anchor biosynthesis
Other DBs Organism Homo sapiens (human) [GN:
hsa ]
Gene 10026 PIGK; phosphatidylinositol glycan anchor biosynthesis class K [KO:K05290 ]
128869 PIGU; phosphatidylinositol glycan anchor biosynthesis class U [KO:K05293 ]
23556 PIGN; phosphatidylinositol glycan anchor biosynthesis class N [KO:K05285 ] [EC:2.7.-.-]
27315 PGAP2; post-GPI attachment to proteins 2 [KO:K23552 ]
2822 GPLD1; glycosylphosphatidylinositol specific phospholipase D1 [KO:K01127 ] [EC:3.1.4.50 ]
284098 PIGW; phosphatidylinositol glycan anchor biosynthesis class W [KO:K05283 ] [EC:2.3.-.-]
51227 PIGP; phosphatidylinositol glycan anchor biosynthesis class P [KO:K03861 ]
51604 PIGT; phosphatidylinositol glycan anchor biosynthesis class T [KO:K05292 ]
5279 PIGC; phosphatidylinositol glycan anchor biosynthesis class C [KO:K03859 ]
5281 PIGF; phosphatidylinositol glycan anchor biosynthesis class F [KO:K05287 ]
5283 PIGH; phosphatidylinositol glycan anchor biosynthesis class H [KO:K03858 ]
54872 PIGG; phosphatidylinositol glycan anchor biosynthesis class G (EMM blood group) [KO:K05310 ] [EC:2.7.-.-]
54965 PIGX; phosphatidylinositol glycan anchor biosynthesis class X [KO:K07541 ]
55650 PIGV; phosphatidylinositol glycan anchor biosynthesis class V [KO:K07542 ] [EC:2.4.1.-]
80055 PGAP1; post-GPI attachment to proteins inositol deacylase 1 [KO:K05294 ] [EC:3.-.-.-]
80235 PIGZ; phosphatidylinositol glycan anchor biosynthesis class Z [KO:K08098 ] [EC:2.4.1.-]
84302 PGAP4; post-GPI attachment to proteins GalNAc transferase 4 [KO:K26235 ]
84720 PIGO; phosphatidylinositol glycan anchor biosynthesis class O [KO:K05288 ] [EC:2.7.-.-]
84992 PIGY; phosphatidylinositol glycan anchor biosynthesis class Y [KO:K11001 ]
8733 GPAA1; glycosylphosphatidylinositol anchor attachment 1 [KO:K05289 ]
8818 DPM2; dolichyl-phosphate mannosyltransferase subunit 2, regulatory [KO:K09658 ]
9091 PIGQ; phosphatidylinositol glycan anchor biosynthesis class Q [KO:K03860 ]
93183 PIGM; phosphatidylinositol glycan anchor biosynthesis class M [KO:K05284 ] [EC:2.4.1.-]
93210 PGAP3; post-GPI attachment to proteins phospholipase 3 [KO:K23553 ]
94005 PIGS; phosphatidylinositol glycan anchor biosynthesis class S [KO:K05291 ]
9487 PIGL; phosphatidylinositol glycan anchor biosynthesis class L [KO:K03434 ] [EC:3.5.1.89 ]
9488 PIGB; phosphatidylinositol glycan anchor biosynthesis class B [KO:K05286 ] [EC:2.4.1.-]
Compound C00350 Phosphatidylethanolamine
C01194 1-Phosphatidyl-D-myo-inositol
G10610 UDP-N-acetyl-D-glucosamine
G10617 Dolichyl phosphate D-mannose
G12396 6-(alpha-D-glucosaminyl)-1D-myo-inositol
Reference Authors Eisenhaber B, Maurer-Stroh S, Novatchkova M, Schneider G, Eisenhaber F.
Title Enzymes and auxiliary factors for GPI lipid anchor biosynthesis and post-translational transfer to proteins.
Journal Reference Authors Kinoshita T, Inoue N.
Title Dissecting and manipulating the pathway for glycosylphos-phatidylinositol-anchor biosynthesis.
Journal Reference Authors Fankhauser C, Homans SW, Thomas-Oates JE, McConville MJ, Desponds C, Conzelmann A, Ferguson MA.
Title Structures of glycosylphosphatidylinositol membrane anchors from Saccharomyces cerevisiae.
Journal J Biol Chem 268:26365-74 (1993)
Reference Authors Murakami Y, Siripanyapinyo U, Hong Y, Kang JY, Ishihara S, Nakakuma H, Maeda Y, Kinoshita T.
Title PIG-W is critical for inositol acylation but not for flipping of glycosylphosphatidylinositol-anchor.
Journal Reference Authors Benachour A, Sipos G, Flury I, Reggiori F, Canivenc-Gansel E, Vionnet C, Conzelmann A, Benghezal M.
Title Deletion of GPI7, a yeast gene required for addition of a side chain to the glycosylphosphatidylinositol (GPI) core structure, affects GPI protein transport, remodeling, and cell wall integrity.
Journal Reference Authors Tanaka S, Maeda Y, Tashima Y, Kinoshita T.
Title Inositol deacylation of glycosylphosphatidylinositol-anchored proteins is mediated by mammalian PGAP1 and yeast Bst1p.
Journal Reference Authors Hong Y, Ohishi K, Kang JY, Tanaka S, Inoue N, Nishimura J, Maeda Y, Kinoshita T.
Title Human PIG-U and yeast Cdc91p are the fifth subunit of GPI transamidase that attaches GPI-anchors to proteins.
Journal Reference Authors Tanaka S, Maeda Y, Kinoshita T.
Title [Biosynthesis of GPI-anchored proteins]
Journal Tanpakushitsu Kakusan Koso 48:990-6 (2003)
Reference Authors Kang JY, Hong Y, Ashida H, Shishioh N, Murakami Y, Morita YS, Maeda Y, Kinoshita T.
Title PIG-V involved in transferring the second mannose in glycosylphosphatidylinositol.
Journal Reference Authors Ashida H, Hong Y, Murakami Y, Shishioh N, Sugimoto N, Kim YU, Maeda Y, Kinoshita T.
Title Mammalian PIG-X and yeast Pbn1p are the essential components of glycosylphosphatidylinositol-mannosyltransferase I.
Journal Reference Authors Fujita M
Title [Biosynthesis and remodeling of GPI-anchored proteins].
Journal Seikagaku 85:985-95 (2013)
Reference Authors Kinoshita T
Title [Biochemistry of glycosylphosphatidylinositol (GPI) anchored proteins].
Journal Seikagaku 86:626-36 (2014)
Reference Authors Fujita M, Maeda Y, Ra M, Yamaguchi Y, Taguchi R, Kinoshita T
Title GPI glycan remodeling by PGAP5 regulates transport of GPI-anchored proteins from the ER to the Golgi.
Journal Reference Authors Kinoshita T, Fujita M
Title Biosynthesis of GPI-anchored proteins: special emphasis on GPI lipid remodeling.
Journal Reference Authors Yang G, Banfield DK.
Title Cdc1p is a Golgi-localized glycosylphosphatidylinositol-anchored protein remodelase.
Journal Reference Authors Kinoshita T.
Title Biosynthesis and biology of mammalian GPI-anchored proteins.
Journal Related KO pathway
