KEGG   PATHWAY: hsa00563
Entry
hsa00563                    Pathway                                
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 metabolism
Pathway map
hsa00563  Glycosylphosphatidylinositol (GPI)-anchor biosynthesis
hsa00563

Module
hsa_M00065  GPI-anchor biosynthesis, core oligosaccharide [PATH:hsa00563]
Network
nt06018  GPI-anchor biosynthesis
  Element
N00748  GPI-anchor biosynthesis
Other DBs
GO: 0006506
Organism
Homo sapiens (human) [GN:hsa]
Gene
5277  PIGA; phosphatidylinositol glycan anchor biosynthesis class A [KO:K03857] [EC:2.4.1.198]
5279  PIGC; phosphatidylinositol glycan anchor biosynthesis class C [KO:K03859]
5283  PIGH; phosphatidylinositol glycan anchor biosynthesis class H [KO:K03858]
51227  PIGP; phosphatidylinositol glycan anchor biosynthesis class P [KO:K03861]
9091  PIGQ; phosphatidylinositol glycan anchor biosynthesis class Q [KO:K03860]
84992  PIGY; phosphatidylinositol glycan anchor biosynthesis class Y [KO:K11001]
8818  DPM2; dolichyl-phosphate mannosyltransferase subunit 2, regulatory [KO:K09658]
9487  PIGL; phosphatidylinositol glycan anchor biosynthesis class L [KO:K03434] [EC:3.5.1.89]
284098  PIGW; phosphatidylinositol glycan anchor biosynthesis class W [KO:K05283] [EC:2.3.-.-]
2822  GPLD1; glycosylphosphatidylinositol specific phospholipase D1 [KO:K01127] [EC:3.1.4.50]
93183  PIGM; phosphatidylinositol glycan anchor biosynthesis class M [KO:K05284] [EC:2.4.1.-]
54965  PIGX; phosphatidylinositol glycan anchor biosynthesis class X [KO:K07541]
55650  PIGV; phosphatidylinositol glycan anchor biosynthesis class V [KO:K07542] [EC:2.4.1.-]
23556  PIGN; phosphatidylinositol glycan anchor biosynthesis class N [KO:K05285] [EC:2.7.-.-]
9488  PIGB; phosphatidylinositol glycan anchor biosynthesis class B [KO:K05286] [EC:2.4.1.-]
84720  PIGO; phosphatidylinositol glycan anchor biosynthesis class O [KO:K05288] [EC:2.7.-.-]
5281  PIGF; phosphatidylinositol glycan anchor biosynthesis class F [KO:K05287]
54872  PIGG; phosphatidylinositol glycan anchor biosynthesis class G [KO:K05310] [EC:2.7.-.-]
8733  GPAA1; glycosylphosphatidylinositol anchor attachment 1 [KO:K05289]
10026  PIGK; phosphatidylinositol glycan anchor biosynthesis class K [KO:K05290]
94005  PIGS; phosphatidylinositol glycan anchor biosynthesis class S [KO:K05291]
51604  PIGT; phosphatidylinositol glycan anchor biosynthesis class T [KO:K05292]
128869  PIGU; phosphatidylinositol glycan anchor biosynthesis class U [KO:K05293]
80055  PGAP1; post-GPI attachment to proteins inositol deacylase 1 [KO:K05294] [EC:3.-.-.-]
65258  MPPE1; metallophosphoesterase 1 [KO:K23362] [EC:3.1.-.-]
80235  PIGZ; phosphatidylinositol glycan anchor biosynthesis class Z [KO:K08098] [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
hsa00510  N-Glycan biosynthesis
hsa00564  Glycerophospholipid metabolism
KO pathway
ko00563   

  All links  
Network (1)   
   KEGG NETWORK (1)   
Genome (1)   
   KEGG GENOME (1)   
Gene (26)   
   KEGG GENES (26)   
All databases (28)   

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