B4GALTs transfer Gal to the keratan chain

Stable Identifier
Homo sapiens
Locations in the PathwayBrowser

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).

Literature References
PubMed ID Title Journal Year
11901181 Deficiency of UDP-galactose:N-acetylglucosamine beta-1,4-galactosyltransferase I causes the congenital disorder of glycosylation type IId

Hansske, B, Thiel, C, Lübke, T, Hasilik, M, Höning, S, Peters, V, Heidemann, PH, Hoffmann, GF, Berger, EG, von Figura, K, Körner, C

J Clin Invest 2002
17021253 Transition of galactosyltransferase 1 from trans-Golgi cisterna to the trans-Golgi network is signal mediated

Schaub, BE, Berger, B, Berger, EG, Rohrer, J

Mol Biol Cell 2006
11588157 Galactosylation of N-linked oligosaccharides by human beta-1,4-galactosyltransferases I, II, III, IV, V, and VI expressed in Sf-9 cells

Guo, S, Sato, T, Shirane, K, Furukawa, K

Glycobiology 2001
9597550 The expanding beta 4-galactosyltransferase gene family: messages from the databanks

Lo, NW, Shaper, JH, Pevsner, J, Shaper, NL

Glycobiology 1998
1714903 Evidence for a molecular distinction between Golgi and cell surface forms of beta 1,4-galactosyltransferase

Lopez, LC, Youakim, A, Evans, SC, Shur, BD

J Biol Chem 1991
Participant Of
Catalyst Activity
Catalyst Activity
beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase activity of B4GALT1-6 homodimers [Golgi membrane]
Physical Entity
Orthologous Events