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2.4.1.134: galactosylxylosylprotein 3-beta-galactosyltransferase

This is an abbreviated version!
For detailed information about galactosylxylosylprotein 3-beta-galactosyltransferase, go to the full flat file.

Word Map on EC 2.4.1.134

Reaction

UDP-alpha-D-galactose
+
[protein]-3-O-(beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine
=
UDP
 +
[protein]-3-O-(beta-D-galactosyl-(1->3)-beta-D-galactosyl-(1->4)-beta-D-xylosyl)-L-serine

Synonyms

B3GALT6, beta3GalT I, beta3GalT II, beta3GalT IV, beta3GalT V, beta3GalT VI, beta3GalT6, dbeta3GalTII, galactosyltransferase II, galactosyltransferase, uridine diphosphogalactose-galactosylxylose, galactosyltransferase-2, GalT2, UDPgalactose:4-beta-D-galactosyl-O-beta-D-xylosylprotein 3-beta-D-galactosyltransferase

ECTree

     2 Transferases
         2.4 Glycosyltransferases
             2.4.1 Hexosyltransferases
                2.4.1.134 galactosylxylosylprotein 3-beta-galactosyltransferase

Engineering

Engineering on EC 2.4.1.134 - galactosylxylosylprotein 3-beta-galactosyltransferase

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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
A108G
naturally occuring enzyme mutation
D207H
naturally occuring enzyme mutation
G217S
naturally occuring enzyme mutation
additional information
homozygosity mapping and candidate gene sequence analysis in three independent families identified biallelic gene B3GALT6 mutations, including homozygous missense mutations in family 1 (c.619G>C [p.Asp207His]) and family 3 (c.649G>A [p.Gly217Ser]) and compound heterozygous mutations in family 2 (c.323_344del [p.Ala108Glyfs*163], c.619G>C [p.Asp207His]), phenotypes overview