Entry |
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Name |
Glycosylphosphatidylinositol (GPI)-anchor biosynthesis |
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.
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Class |
Metabolism; Glycan biosynthesis and metabolism
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Pathway map |
rn00563 | Glycosylphosphatidylinositol (GPI)-anchor biosynthesis |

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Module |
M00065 | GPI-anchor biosynthesis, core oligosaccharide [PATH:rn00563] |
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Other DBs |
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Reaction |
R06623 | glycoprotein-phosphatidylinositol phosphatidohydrolase |
R13391 | UDP-galactose:beta-N-acetyl-galactosamine-beta-1,3-galactosyltransferase |
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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 |
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Reference |
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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 |
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Reference |
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Authors |
Kinoshita T, Inoue N. |
Title |
Dissecting and manipulating the pathway for glycosylphos-phatidylinositol-anchor biosynthesis. |
Journal |
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Reference |
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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 |
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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 |
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Reference |
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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 |
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Reference |
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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 |
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Reference |
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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 |
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Reference |
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Authors |
Tanaka S, Maeda Y, Kinoshita T. |
Title |
[Biosynthesis of GPI-anchored proteins] |
Journal |
Tanpakushitsu Kakusan Koso 48:990-6 (2003) |
Reference |
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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 |
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Reference |
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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 |
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Reference |
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Authors |
Fujita M |
Title |
[Biosynthesis and remodeling of GPI-anchored proteins]. |
Journal |
Seikagaku 85:985-95 (2013) |
Reference |
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Authors |
Kinoshita T |
Title |
[Biochemistry of glycosylphosphatidylinositol (GPI) anchored proteins]. |
Journal |
Seikagaku 86:626-36 (2014) |
Reference |
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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 |
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Reference |
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Authors |
Kinoshita T, Fujita M |
Title |
Biosynthesis of GPI-anchored proteins: special emphasis on GPI lipid remodeling. |
Journal |
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Reference |
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Authors |
Yang G, Banfield DK. |
Title |
Cdc1p is a Golgi-localized glycosylphosphatidylinositol-anchored protein remodelase. |
Journal |
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Reference |
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Authors |
Kinoshita T. |
Title |
Biosynthesis and biology of mammalian GPI-anchored proteins. |
Journal |
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Related pathway |
rn00564 | Glycerophospholipid metabolism |
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KO pathway |
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