KEGG   PATHWAY: rn00515
Entry
rn00515                     Pathway                                
Name
Mannose type O-glycan biosynthesis
Description
Biosynthesis of mammalian O-mannosyl glycans is initiated by the transfer of mannose from mannose-P-Dol to serine or threonine residue, followed by extensions with N-acetylglucosamine (GlcNAc) and galactose (Gal) to generate core M1, M2 and M3 glycans. Core M1 and M2 glycans can then be further attached by fucose residues, sialic acid terminals and sulfatded glucuroinc acid terminals. Core M3 glycan is involved in the synthesis of alpha-dystroglycan, a heavily glycosylated protein found in muscle and brain tissues. Core M3 glycan contains a tandem repeat of ribitol 5-phosphate (Rbo5P) and -alpha3-GlcA-beta3-Xyl- repeating structures. Defects of genes encoding core glycans and modified core M3 glycans are associated with various congenital diseases, such as muscular dystrophies caused by reduced O-mannosylation of alpha-dystroglycan in skeletal muscles [DS:H00120].
Class
Metabolism; Glycan biosynthesis and metabolism
Pathway map
rn00515  Mannose type O-glycan biosynthesis
rn00515

Module
M00872  O-glycan biosynthesis, mannose type (core M3) [PATH:rn00515]
Other DBs
GO: 0035269
Reaction
R02921  CTP:D-ribitol-5-phosphate cytidylyltransferase
R07619   
R07620   
R07621   
R11407   
R12244  UDP-alpha-D-xylose:3-O-[Rib-ol-P-Rib-ol-P-3-beta-D-GalNAc-(1->3)-beta-D-GlcNAc-(1->4)-O-6-P-alpha-D-Man]-Ser/Thr-[protein] xylosyltransferase
R12292   
R12293   
R12294   
R12295   
R12296   
R12297   
R12298   
Compound
C00063  CTP
C00789  CDP-ribitol
C01068  D-Ribitol 5-phosphate
C02189  [Protein]-L-serine
G10617  Dolichyl phosphate D-mannose
G13026   
G13027   
G13028   
G13082   
G13083   
G13084  O-Mannose core M1
G13085   
G13086  O-Mannose core M2
G13087   
G13088   
G13089   
G13090   
G13091  O-mannose core M3
G13092  Phospho-core M3
G13093   
G13094   
G13095   
G13096   
G13097   
G13098   
G13099   
G13100   
Reference
  Authors
Praissman JL, Wells L
  Title
Mammalian O-mannosylation pathway: glycan structures, enzymes, and protein substrates.
  Journal
Biochemistry 53:3066-78 (2014)
DOI:10.1021/bi500153y
Reference
  Authors
Stalnaker SH, Stuart R, Wells L
  Title
Mammalian O-mannosylation: unsolved questions of structure/function.
  Journal
Curr Opin Struct Biol 21:603-9 (2011)
DOI:10.1016/j.sbi.2011.09.001
Reference
  Authors
Kanagawa M, Kobayashi K, Tajiri M, Manya H, Kuga A, Yamaguchi Y, Akasaka-Manya K, Furukawa J, Mizuno M, Kawakami H, Shinohara Y, Wada Y, Endo T, Toda T
  Title
Identification of a Post-translational Modification with Ribitol-Phosphate and Its Defect in Muscular Dystrophy.
  Journal
Cell Rep 14:2209-23 (2016)
DOI:10.1016/j.celrep.2016.02.017
Reference
  Authors
Manya H, Yamaguchi Y, Kanagawa M, Kobayashi K, Tajiri M, Akasaka-Manya K, Kawakami H, Mizuno M, Wada Y, Toda T, Endo T
  Title
The Muscular Dystrophy Gene TMEM5 Encodes a Ribitol beta1,4-Xylosyltransferase Required for the Functional Glycosylation of Dystroglycan.
  Journal
J Biol Chem 291:24618-24627 (2016)
DOI:10.1074/jbc.M116.751917
Related
pathway
rn00510  N-Glycan biosynthesis
KO pathway
ko00515   
LinkDB

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