Entry Name Glycosylphosphatidylinositol (GPI)-anchor biosynthesis - Ursus arctos horribilis (North American brown bear)
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 uah00563 Glycosylphosphatidylinositol (GPI)-anchor biosynthesis
Ortholog table Module Other DBs Organism Ursus arctos horribilis (North American brown bear) [GN:
uah ]
Gene 113262828 PIGC; phosphatidylinositol N-acetylglucosaminyltransferase subunit C [KO:K03859 ]
113248302 phosphatidylinositol N-acetylglucosaminyltransferase subunit H isoform X1 [KO:K03858 ]
113248316 phosphatidylinositol N-acetylglucosaminyltransferase subunit H isoform X1 [KO:K03858 ]
113259656 PIGP; phosphatidylinositol N-acetylglucosaminyltransferase subunit P isoform X1 [KO:K03861 ]
113245379 PIGQ; phosphatidylinositol N-acetylglucosaminyltransferase subunit Q isoform X1 [KO:K03860 ]
113263154 PIGY; phosphatidylinositol N-acetylglucosaminyltransferase subunit Y [KO:K11001 ]
113266448 DPM2; dolichol phosphate-mannose biosynthesis regulatory protein isoform X1 [KO:K09658 ]
113268914 PIGW; phosphatidylinositol-glycan biosynthesis class W protein [KO:K05283 ] [EC:2.3.-.-]
113242811 PIGX; phosphatidylinositol-glycan biosynthesis class X protein [KO:K07541 ]
113253478 PIGN; GPI ethanolamine phosphate transferase 1 [KO:K05285 ] [EC:2.7.-.-]
113269648 PIGB; GPI mannosyltransferase 3 isoform X1 [KO:K05286 ] [EC:2.4.1.-]
113260489 PIGO; GPI ethanolamine phosphate transferase 3 isoform X1 [KO:K05288 ] [EC:2.7.-.-]
113246082 PIGF; phosphatidylinositol-glycan biosynthesis class F protein [KO:K05287 ]
113245729 PIGG; GPI ethanolamine phosphate transferase 2 isoform X1 [KO:K05310 ] [EC:2.7.-.-]
113247675 GPAA1; glycosylphosphatidylinositol anchor attachment 1 protein [KO:K05289 ]
113258516 PIGU; phosphatidylinositol glycan anchor biosynthesis class U protein isoform X1 [KO:K05293 ]
113250635 PGAP1; GPI inositol-deacylase isoform X1 [KO:K05294 ] [EC:3.-.-.-]
113245923 MPPE1; metallophosphoesterase 1 isoform X1 [KO:K23362 ] [EC:3.1.-.-]
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 Related KO pathway LinkDB All DBs
