KEGG   PATHWAY: hsa00510Help
Entry
hsa00510                    Pathway                                

Name
N-Glycan biosynthesis - Homo sapiens (human)
Description
N-glycans or asparagine-linked glycans are major constituents of glycoproteins in eukaryotes. N-glycans are covalently attached to asparagine with the consensus sequence of Asn-X-Ser/Thr by an N-glycosidic bond, GlcNAc b1- Asn. Biosynthesis of N-glycans begins on the cytoplasmic face of the ER membrane with the transferase reaction of UDP-GlcNAc and the lipid-like precursor P-Dol (dolichol phosphate) to generate GlcNAc a1- PP-Dol. After sequential addition of monosaccharides by ALG glycosyltransferases [MD:M00055], the N-glycan precursor is attached by the OST (oligosaccharyltransferase) complex to the polypeptide chain that is being synthesized and translocated through the ER membrane. The protein-bound N-glycan precursor is subsequently trimmed, extended, and modified in the ER and Golgi by a complex series of reactions catalyzed by membrane-bound glycosidases and glycosyltransferases. N-glycans thus synthesized are classified into three types: high-mannose type, complex type, and hybrid type. Defects in N-glycan biosynthesis lead to a variety of human diseases known as congenital disorders of glycosylation [DS:H00118 H00119].
Class
Metabolism; Glycan biosynthesis and metabolism
BRITE hierarchy
Pathway map
hsa00510  N-Glycan biosynthesis
hsa00510

Ortholog table
Module
hsa_M00055  N-glycan precursor biosynthesis [PATH:hsa00510]
hsa_M00072  N-glycosylation by oligosaccharyltransferase [PATH:hsa00510]
hsa_M00073  N-glycan precursor trimming [PATH:hsa00510]
hsa_M00075  N-glycan biosynthesis, complex type [PATH:hsa00510]
Disease
H00118  Congenital disorders of glycosylation (CDG) type I
H00119  Congenital disorders of glycosylation (CDG) type II
Other DBs
BSID: 82975
GO: 0006487
Organism
Homo sapiens (human) [GN:hsa]
Gene
22845  DOLK; dolichol kinase [KO:K00902] [EC:2.7.1.108]
1798  DPAGT1; dolichyl-phosphate N-acetylglucosaminephosphotransferase 1 [KO:K01001] [EC:2.7.8.15]
29880  ALG5; ALG5, dolichyl-phosphate beta-glucosyltransferase [KO:K00729] [EC:2.4.1.117]
79868  ALG13; ALG13, UDP-N-acetylglucosaminyltransferase subunit [KO:K07432] [EC:2.4.1.141]
199857  ALG14; ALG14, UDP-N-acetylglucosaminyltransferase subunit [KO:K07441] [EC:2.4.1.141]
8813  DPM1; dolichyl-phosphate mannosyltransferase subunit 1, catalytic [KO:K00721] [EC:2.4.1.83]
8818  DPM2; dolichyl-phosphate mannosyltransferase subunit 2, regulatory [KO:K09658]
54344  DPM3; dolichyl-phosphate mannosyltransferase subunit 3 [KO:K09659]
56052  ALG1; ALG1, chitobiosyldiphosphodolichol beta-mannosyltransferase [KO:K03842] [EC:2.4.1.142]
85365  ALG2; ALG2, alpha-1,3/1,6-mannosyltransferase [KO:K03843] [EC:2.4.1.257 2.4.1.132]
440138  ALG11; ALG11, alpha-1,2-mannosyltransferase [KO:K03844] [EC:2.4.1.131]
10195  ALG3; ALG3, alpha-1,3- mannosyltransferase [KO:K03845] [EC:2.4.1.258]
79796  ALG9; ALG9, alpha-1,2-mannosyltransferase [KO:K03846] [EC:2.4.1.261 2.4.1.259]
79087  ALG12; ALG12, alpha-1,6-mannosyltransferase [KO:K03847] [EC:2.4.1.260]
29929  ALG6; ALG6, alpha-1,3-glucosyltransferase [KO:K03848] [EC:2.4.1.267]
79053  ALG8; ALG8, alpha-1,3-glucosyltransferase [KO:K03849] [EC:2.4.1.265]
84920  ALG10; ALG10, alpha-1,2-glucosyltransferase [KO:K03850] [EC:2.4.1.256]
144245  ALG10B; ALG10B, alpha-1,2-glucosyltransferase [KO:K03850] [EC:2.4.1.256]
3703  STT3A; STT3A, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
201595  STT3B; STT3B, catalytic subunit of the oligosaccharyltransferase complex [KO:K07151] [EC:2.4.99.18]
6184  RPN1; ribophorin I [KO:K12666]
6185  RPN2; ribophorin II [KO:K12667]
1603  DAD1; defender against cell death 1 [KO:K12668]
7991  TUSC3; tumor suppressor candidate 3 [KO:K12669]
1650  DDOST; dolichyl-diphosphooligosaccharide--protein glycosyltransferase non-catalytic subunit [KO:K12670]
57171  DOLPP1; dolichyldiphosphatase 1 [KO:K07252] [EC:3.6.1.43]
7841  MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
23193  GANAB; glucosidase II alpha subunit [KO:K05546] [EC:3.2.1.207]
11253  MAN1B1; mannosidase alpha class 1B member 1 [KO:K01230] [EC:3.2.1.113]
10905  MAN1A2; mannosidase alpha class 1A member 2 [KO:K01230] [EC:3.2.1.113]
57134  MAN1C1; mannosidase alpha class 1C member 1 [KO:K01230] [EC:3.2.1.113]
4121  MAN1A1; mannosidase alpha class 1A member 1 [KO:K01230] [EC:3.2.1.113]
4245  MGAT1; mannosyl (alpha-1,3-)-glycoprotein beta-1,2-N-acetylglucosaminyltransferase [KO:K00726] [EC:2.4.1.101]
4124  MAN2A1; mannosidase alpha class 2A member 1 [KO:K01231] [EC:3.2.1.114]
4122  MAN2A2; mannosidase alpha class 2A member 2 [KO:K01231] [EC:3.2.1.114]
4247  MGAT2; mannosyl (alpha-1,6-)-glycoprotein beta-1,2-N-acetylglucosaminyltransferase [KO:K00736] [EC:2.4.1.143]
2530  FUT8; fucosyltransferase 8 [KO:K00717] [EC:2.4.1.68]
2683  B4GALT1; beta-1,4-galactosyltransferase 1 [KO:K07966] [EC:2.4.1.- 2.4.1.38 2.4.1.90 2.4.1.22]
8704  B4GALT2; beta-1,4-galactosyltransferase 2 [KO:K07967] [EC:2.4.1.- 2.4.1.38 2.4.1.90 2.4.1.22]
8703  B4GALT3; beta-1,4-galactosyltransferase 3 [KO:K07968] [EC:2.4.1.- 2.4.1.38 2.4.1.90]
6480  ST6GAL1; ST6 beta-galactoside alpha-2,6-sialyltransferase 1 [KO:K00778] [EC:2.4.99.1]
84620  ST6GAL2; ST6 beta-galactoside alpha-2,6-sialyltransferase 2 [KO:K00779] [EC:2.4.99.1]
4248  MGAT3; mannosyl (beta-1,4-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase [KO:K00737] [EC:2.4.1.144]
11320  MGAT4A; mannosyl (alpha-1,3-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, isozyme A [KO:K00738] [EC:2.4.1.145]
11282  MGAT4B; mannosyl (alpha-1,3-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, isozyme B [KO:K00738] [EC:2.4.1.145]
152586  MGAT4D; MGAT4 family member D [KO:K00738] [EC:2.4.1.145]
4249  MGAT5; mannosyl (alpha-1,6-)-glycoprotein beta-1,6-N-acetyl-glucosaminyltransferase [KO:K00744] [EC:2.4.1.155]
146664  MGAT5B; mannosyl (alpha-1,6-)-glycoprotein beta-1,6-N-acetyl-glucosaminyltransferase, isozyme B [KO:K09661] [EC:2.4.1.-]
25834  MGAT4C; MGAT4 family member C [KO:K13748] [EC:2.4.1.145 2.4.1.201]
Compound
C00096  GDP-mannose
C00110  Dolichyl phosphate
C00381  Dolichol
C00621  Dolichyl diphosphate
C01246  Dolichyl beta-D-glucosyl phosphate
C03021  Protein asparagine
C03862  Dolichyl phosphate D-mannose
G00001  N-Acetyl-D-glucosaminyldiphosphodolichol
G00002  N,N'-Chitobiosyldiphosphodolichol
G00003   
G00004   
G00005   
G00006   
G00007   
G00008   
G00009   
G00010   
G00011   
G00012   
G00013   
G00014   
G00015   
G00016   
G00017   
G00018  DS 3
G00019   
G00020   
G00021   
G00022   
G00171   
G10526   
G10595   
G10596   
G10597   
G10598   
G10599   
Reference
PMID:9111134
  Authors
Schutzbach JS.
  Title
The role of the lipid matrix in the biosynthesis of dolichyl-linked oligosaccharides.
  Journal
Glycoconj J 14:175-82 (1997)
DOI:10.1023/A:1018533620002
Reference
  Authors
Oriol R, Martinez-Duncker I, Chantret I, Mollicone R, Codogno P.
  Title
Common origin and evolution of glycosyltransferases using Dol-P-monosaccharides as donor substrate.
  Journal
Mol Biol Evol 19:1451-63 (2002)
Reference
  Authors
Thiel C, Schwarz M, Peng J, Grzmil M, Hasilik M, Braulke T, Kohlschutter A, von Figura K, Lehle L, Korner C.
  Title
A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis.
  Journal
J Biol Chem 278:22498-505 (2003)
DOI:10.1074/jbc.M302850200
Reference
  Authors
Cipollo JF, Trimble RB, Chi JH, Yan Q, Dean N.
  Title
The yeast ALG11 gene specifies addition of the terminal alpha 1,2-Man to the Man5GlcNAc2-PP-dolichol N-glycosylation intermediate formed on the cytosolic side of the endoplasmic reticulum.
  Journal
J Biol Chem 276:21828-40 (2001)
DOI:10.1074/jbc.M010896200
Reference
  Authors
Frank CG, Aebi M.
  Title
ALG9 mannosyltransferase is involved in two different steps of lipid-linked oligosaccharide biosynthesis.
  Journal
Glycobiology 15:1156-63 (2005)
DOI:10.1093/glycob/cwj002
Reference
  Authors
O'Reilly MK, Zhang G, Imperiali B.
  Title
In vitro evidence for the dual function of Alg2 and Alg11: essential mannosyltransferases in N-linked glycoprotein biosynthesis.
  Journal
Biochemistry 45:9593-603 (2006)
DOI:10.1021/bi060878o
KO pathway
ko00510   
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