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
N-Glycan biosynthesis
hsa00510

All organismsOrtholog table
Module
N-glycan precursor biosynthesis [PATH:hsa00510]
Oligosaccharyltransferase [PATH:hsa00510]
N-glycan precursor trimming [PATH:hsa00510]
N-glycan biosynthesis, high-mannose type [PATH:hsa00510]
N-glycan biosynthesis, complex type [PATH:hsa00510]
Disease
H00118  
Congenital disorders of glycosylation (CDG) type I
H00119  
Congenital disorders of glycosylation (CDG) type II
H00768  
Nonsyndromic autosomal recessive mental retardation (NS-ARMR)
Other DBs
BSID: 
GO: 
Organism
Homo sapiens (human) [GN:hsa]
Gene
22845  
DOLK; dolichol kinase [KO:K00902] [EC:2.7.1.108]
1798  
DPAGT1; dolichyl-phosphate (UDP-N-acetylglucosamine) N-acetylglucosaminephosphotransferase 1 (GlcNAc-1-P transferase) [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 polypeptide 1, catalytic subunit [KO:K00721] [EC:2.4.1.83]
8818  
DPM2; dolichyl-phosphate mannosyltransferase polypeptide 2, regulatory subunit [KO:K09658]
54344  
DPM3; dolichyl-phosphate mannosyltransferase polypeptide 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]
91869  
RFT1; RFT1 homolog (S. cerevisiae) [KO:K06316]
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, subunit of the oligosaccharyltransferase complex (catalytic) [KO:K07151] [EC:2.4.99.18]
201595  
STT3B; STT3B, subunit of the oligosaccharyltransferase complex (catalytic) [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 subunit (non-catalytic) [KO:K12670]
57171  
DOLPP1; dolichyl pyrophosphate phosphatase 1 [KO:K07252] [EC:3.6.1.43]
7841  
MOGS; mannosyl-oligosaccharide glucosidase [KO:K01228] [EC:3.2.1.106]
23193  
GANAB; glucosidase, alpha [KO:K05546] [EC:3.2.1.84]
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 (alpha (1,6) fucosyltransferase) [KO:K00717] [EC:2.4.1.68]
2683  
B4GALT1; UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1 [KO:K07966] [EC:2.4.1.- 2.4.1.38 2.4.1.90 2.4.1.22]
8704  
B4GALT2; UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2 [KO:K07967] [EC:2.4.1.- 2.4.1.38 2.4.1.90 2.4.1.22]
8703  
B4GALT3; UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3 [KO:K07968] [EC:2.4.1.- 2.4.1.38 2.4.1.90]
6480  
ST6GAL1; ST6 beta-galactosamide alpha-2,6-sialyltranferase 1 [KO:K00778] [EC:2.4.99.1]
84620  
ST6GAL2; ST6 beta-galactosamide alpha-2,6-sialyltranferase 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]
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; mannosyl (alpha-1,3-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, isozyme C (putative) [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)
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)
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)
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)
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)
KO pathway
 
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