KEGG   PATHWAY: hsa00514Help
Entry
hsa00514                    Pathway                                

Name
Other types of O-glycan biosynthesis - Homo sapiens (human)
Description
O-mannosyl glycans are a type of O-glycans that are found both in eukaryotes and prokaryotes. Biosynthesis of O-mannosyl glycans is initiated by the transfer of mannose from Man-P-Dol to serine or threonine residue, which is catalyzed by protein O-mannosyltransferases POMT1 and POMT2. Defects of these genes are linked to human diseases, such as muscular dystrophies caused by reduced O-mannosylation of alpha-dystroglycan in skeletal muscles [DS:H00120].
Class
Metabolism; Glycan biosynthesis and metabolism
BRITE hierarchy
Pathway map
Other types of O-glycan biosynthesis
hsa00514

All organismsOrtholog table
Disease
H00120  
Dystroglycanopathy
H00590  
Congenital muscular dystrophies (CMD/MDC)
H00593  
Limb-girdle muscular dystrophy (LGMD)
H00768  
Nonsyndromic autosomal recessive mental retardation (NS-ARMR)
H00944  
Dowling-Degos disease
H01075  
Peters anomaly
H01192  
Lysyl hydroxylase 3 (LH3) deficiency
Organism
Homo sapiens (human) [GN:hsa]
Gene
10585  
POMT1; protein O-mannosyltransferase 1 [KO:K00728] [EC:2.4.1.109]
29954  
POMT2; protein O-mannosyltransferase 2 [KO:K00728] [EC:2.4.1.109]
55624  
POMGNT1; protein O-linked mannose N-acetylglucosaminyltransferase 1 (beta 1,2-) [KO:K09666] [EC:2.4.1.-]
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]
6487  
ST3GAL3; ST3 beta-galactoside alpha-2,3-sialyltransferase 3 [KO:K00781] [EC:2.4.99.6]
10690  
FUT9; fucosyltransferase 9 (alpha (1,3) fucosyltransferase) [KO:K03663] [EC:2.4.1.152]
2526  
FUT4; fucosyltransferase 4 [KO:K07632] [EC:2.4.1.-]
2529  
FUT7; fucosyltransferase 7 (alpha (1,3) fucosyltransferase) [KO:K07635] [EC:2.4.1.-]
146664  
MGAT5B; mannosyl (alpha-1,6-)-glycoprotein beta-1,6-N-acetyl-glucosaminyltransferase, isozyme B [KO:K09661] [EC:2.4.1.-]
8705  
B3GALT4; Beta-1,3-galactosyltransferase 4 [KO:K00715] [EC:2.4.1.62]
27087  
B3GAT1; beta-1,3-glucuronyltransferase 1 [KO:K00735] [EC:2.4.1.135]
135152  
B3GAT2; beta-1,3-glucuronyltransferase 2 [KO:K10157] [EC:2.4.1.135]
9486  
CHST10; carbohydrate sulfotransferase 10 [KO:K09674] [EC:2.8.2.-]
8473  
OGT; O-linked N-acetylglucosamine (GlcNAc) transferase [KO:K09667] [EC:2.4.1.255]
285203  
EOGT; EGF domain-specific O-linked N-acetylglucosamine (GlcNAc) transferase [KO:K18134] [EC:2.4.1.255]
23509  
POFUT1; protein O-fucosyltransferase 1 [KO:K03691] [EC:2.4.1.221]
23275  
POFUT2; protein O-fucosyltransferase 2 [KO:K03691] [EC:2.4.1.221]
4242  
MFNG; MFNG O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase [KO:K05948] [EC:2.4.1.222]
3955  
LFNG; LFNG O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase [KO:K05948] [EC:2.4.1.222]
5986  
RFNG; RFNG O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase [KO:K05948] [EC:2.4.1.222]
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]
145173  
B3GLCT; beta 3-glucosyltransferase [KO:K13675] [EC:2.4.1.-]
56983  
POGLUT1; protein O-glucosyltransferase 1 [KO:K13667] [EC:2.4.1.-]
283464  
GXYLT1; glucoside xylosyltransferase 1 [KO:K13676] [EC:2.4.2.-]
727936  
GXYLT2; glucoside xylosyltransferase 2 [KO:K13676] [EC:2.4.2.-]
79709  
COLGALT1; collagen beta(1-O)galactosyltransferase 1 [KO:K11703] [EC:2.4.1.50]
23127  
COLGALT2; collagen beta(1-O)galactosyltransferase 2 [KO:K11703] [EC:2.4.1.50]
8985  
PLOD3; procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3 [KO:K13646] [EC:2.4.1.66 2.4.1.50 1.14.11.4]
Reference
  Authors
Kizuka Y, Oka S
  Title
Regulated expression and neural functions of human natural killer-1 (HNK-1) carbohydrate.
  Journal
Cell Mol Life Sci 69:4135-47 (2012)
Reference
  Authors
Lommel M, Strahl S
  Title
Protein O-mannosylation: conserved from bacteria to humans.
  Journal
Glycobiology 19:816-28 (2009)
KO pathway
 

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