KEGG   PATHWAY: map00515
Entry
map00515                    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
map00515  Mannose type O-glycan biosynthesis
map00515

Module
M00872  O-glycan biosynthesis, mannose type (core M3) [PATH:map00515]
Disease
H00120  Muscular dystrophy-dystroglycanopathy type A
H00593  Limb-girdle muscular dystrophy
H01957  Fukuyama congenital muscular dystrophy
H01959  Muscular dystrophy-dystroglycanopathy type C
H01960  Muscular dystrophy-dystroglycanopathy type B
H01961  Congenital muscular dystrophy type 1C
H01962  Congenital muscular dystrophy type 1D
H02307  Muscular dystrophy-dystroglycanopathy
Other DBs
GO: 0006493
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
map00510  N-Glycan biosynthesis
KO pathway
ko00515   

DBGET integrated database retrieval system