The family of beta 4-galactosyltransferases (B4GALTs) is composed of at least six known members with different Km and acceptor specificities (Guo et al. 2001) that probably originated by gene duplication (Lo et al. 1998). They mediate the transfer of galactose to N-glycan structures which initiate the beginning of keratan sulfate (KS) biosynthesis. 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).
Rohrer, J, Berger, B, Berger, EG, Schaub, BE
Hoffmann, GF, Lübke, T, Peters, V, Hansske, B, Körner, C, Heidemann, PH, von Figura, K, Berger, EG, Höning, S, Thiel, C, Hasilik, M
Guo, S, Sato, T, Furukawa, K, Shirane, K
Shur, BD, Lopez, LC, Youakim, A, Evans, SC
Pevsner, J, Shaper, NL, Shaper, JH, Lo, NW
beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase activity of B4GALT1-6 homodimers [Golgi membrane]
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