path: : Syntax Error.
Usage : dbname:identifier

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
  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 (EMM blood group) [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)   

Download RDF
DBGET integrated database retrieval system