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A4GALT

Domain

The conserved DXD motif is involved in enzyme activity.

Function

Catalyzes the transfer of galactose from UDP-alpha-D-galactose to lactosylceramide/beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide(d18:1(4E)) to produce globotriaosylceramide/globoside Gb3Cer (d18:1(4E)) (PubMed:10748143). Also able to transfer galactose to galactosylceramide/beta-D-Gal-(1<->1')-Cer (PubMed:10748143). Globoside Gb3Cer is a glycosphingolipid of the globo serie, one of the major types of neutral root structures of glycosphingolipids, that constitute a significant portion of mammalian cell membranes (Probable). Globotriaosylceramide/globoside Gb3Cer in blood and tissue cell membranes is the antigen Pk of blood histogroup P (PubMed:10747952).

(Microbial infection) Globotriaosylceramide is one of the cellular ligands for bacterial verotoxins.

Pathway

Glycolipid biosynthesis.

Sequence Similarities

Belongs to the glycosyltransferase 32 family.

Tissue Specificity

Ubiquitous. Highly expressed in kidney, heart, spleen, liver, testis and placenta.

Cellular localization

Alternative names

A14GALT, A4GALT1, A4GALT, Lactosylceramide 4-alpha-galactosyltransferase, Alpha4Gal-T1, CD77 synthase, Globotriaosylceramide synthase, P1/Pk synthase, UDP-galactose:beta-D-galactosyl-beta1-R 4-alpha-D-galactosyltransferase, Gb3 synthase

swissprot:Q9NPC4 entrezGene:53947 omim:607922