Reaction: B4GALTs transfer Gal to the keratan chain
- in pathway: Keratan sulfate biosynthesis
The family of beta 4-galactosyltransferases (B4GALTs) is composed by at least six known members with different Km and acceptor specificities (Guo et al. 2001) and probably originated by duplication (Lo et al. 1998). They mediate the transfer of galactose to N-glycan structures, either to begin, or in this case, to elongate keratan chains. B4GALT1 is associated with Congenital Disorder of Glycosylation of type IId (MIM:607091) (Hansske et al. 2002), and is expressed as two splicing isoforms of which only one is localized in the Golgi system (Lopez et al. 1991, Schaub et al. 2006).
Reaction - small molecule participants:
UDP [Golgi lumen]
UDP-Gal [Golgi lumen]
Reactome.org reaction link: R-HSA-2046265
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Reaction input - small molecules:
UDP-alpha-D-galactose(2-)
Reaction output - small molecules:
UDP(3-)
Reactome.org link: R-HSA-2046265