KEGG   PATHWAY: ko00563
Entry
ko00563                     Pathway                                
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.
Class
Metabolism; Glycan biosynthesis and metabolism
Pathway map
ko00563  Glycosylphosphatidylinositol (GPI)-anchor biosynthesis
ko00563

Module
M00065  GPI-anchor biosynthesis, core oligosaccharide [PATH:ko00563]
Drug
D11694  Fosmanogepix (USAN/INN)
Other DBs
GO: 0006506
Orthology
K03857  phosphatidylinositol N-acetylglucosaminyltransferase subunit A [EC:2.4.1.198]
K03859  phosphatidylinositol N-acetylglucosaminyltransferase subunit C
K03858  phosphatidylinositol N-acetylglucosaminyltransferase subunit H
K03861  phosphatidylinositol N-acetylglucosaminyltransferase subunit P
K03860  phosphatidylinositol N-acetylglucosaminyltransferase subunit Q
K11001  phosphatidylinositol N-acetylglucosaminyltransferase subunit Y
K11002  phosphatidylinositol N-acetylglucosaminyltransferase ERI1 subunit
K09658  dolichol phosphate-mannose biosynthesis regulatory protein
K03434  N-acetylglucosaminylphosphatidylinositol deacetylase [EC:3.5.1.89]
K05283  glucosaminylphosphatidylinositol acyltransferase [EC:2.3.-.-]
K01127  glycosylphosphatidylinositol phospholipase D [EC:3.1.4.50]
K05284  GPI mannosyltransferase 1 subunit M [EC:2.4.1.-]
K07541  GPI mannosyltransferase 1 subunit X
K07542  GPI mannosyltransferase 2 [EC:2.4.1.-]
K05285  GPI ethanolamine phosphate transferase 1 [EC:2.7.-.-]
K05286  GPI mannosyltransferase 3 [EC:2.4.1.-]
K05288  GPI ethanolamine phosphate transferase 3 subunit O [EC:2.7.-.-]
K05287  GPI ethanolamine phosphate transferase 2/3 subunit F
K05310  ethanolamine phosphate transferase 2 subunit G [EC:2.7.-.-]
K05289  GPI-anchor transamidase subunit GAA1
K05290  GPI-anchor transamidase subunit K
K05291  GPI-anchor transamidase subunit S
K05292  GPI-anchor transamidase subunit T
K05293  GPI-anchor transamidase subunit U
K05294  GPI inositol-deacylase [EC:3.-.-.-]
K23362  ethanolamine phosphate phosphodiesterase [EC:3.1.-.-]
K08098  GPI mannosyltransferase 4 [EC:2.4.1.-]
Compound
C00154  Palmitoyl-CoA
C00346  Ethanolamine phosphate
C00350  Phosphatidylethanolamine
C01194  1-Phosphatidyl-D-myo-inositol
G00143   
G00144   
G00145   
G00146   
G00148   
G00149   
G10610  UDP-N-acetyl-D-glucosamine
G10617  Dolichyl phosphate D-mannose
G12396  6-(alpha-D-glucosaminyl)-1D-myo-inositol
G13044   
G13046   
G13128   
G13150   
G13151   
G13152   
Reference
PMID:12655644
  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
Bioessays 25:367-85 (2003)
DOI:10.1002/bies.10254
Reference
PMID:11102867
  Authors
Kinoshita T, Inoue N.
  Title
Dissecting and manipulating the pathway for glycosylphos-phatidylinositol-anchor biosynthesis.
  Journal
Curr Opin Chem Biol 4:632-8 (2000)
DOI:10.1016/S1367-5931(00)00151-4
Reference
PMID:8253761
  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
PMID:14517336
  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
Mol Biol Cell 14:4285-95 (2003)
DOI:10.1091/mbc.E03-03-0193
Reference
PMID:10329735
  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
J Biol Chem 274:15251-61 (1999)
DOI:10.1074/jbc.274.21.15251
Reference
PMID:14734546
  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
J Biol Chem 279:14256-63 (2004)
DOI:10.1074/jbc.M313755200
Reference
PMID:12802054
  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
Mol Biol Cell 14:1780-9 (2003)
DOI:10.1091/mbc.E02-12-0794
Reference
PMID:12807000
  Authors
Tanaka S, Maeda Y, Kinoshita T.
  Title
[Biosynthesis of GPI-anchored proteins]
  Journal
Tanpakushitsu Kakusan Koso 48:990-6 (2003)
Reference
PMID:15623507
  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
J Biol Chem 280:9489-97 (2005)
DOI:10.1074/jbc.M413867200
Reference
PMID:15635094
  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
Mol Biol Cell 16:1439-48 (2005)
DOI:10.1091/mbc.E04-09-0802
Reference
PMID:24364251
  Authors
Fujita M
  Title
[Biosynthesis and remodeling of GPI-anchored proteins].
  Journal
Seikagaku 85:985-95 (2013)
Reference
PMID:25509331
  Authors
Kinoshita T
  Title
[Biochemistry of glycosylphosphatidylinositol (GPI) anchored proteins].
  Journal
Seikagaku 86:626-36 (2014)
Reference
PMID:19837036
  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
Cell 139:352-65 (2009)
DOI:10.1016/j.cell.2009.08.040
Reference
PMID:26563290
  Authors
Kinoshita T, Fujita M
  Title
Biosynthesis of GPI-anchored proteins: special emphasis on GPI lipid remodeling.
  Journal
J Lipid Res 57:6-24 (2016)
DOI:10.1194/jlr.R063313
Related
pathway
ko00510  N-Glycan biosynthesis
ko00564  Glycerophospholipid metabolism

  All links  
Gene (27)   
   KEGG ORTHOLOGY (27)   
All databases (27)   

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