LMPD Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
LMP006537

UniProt Annotations

Entry Information
Gene Namealpha 1,3-galactosyltransferase 2 (isoglobotriaosylceramide synthase)
Protein EntryA3LT2_MOUSE
UniProt IDQ3V1N9
SpeciesMouse
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q3V1N9-1; Sequence=Displayed; Name=2; IsoId=Q3V1N9-2; Sequence=VSP_054297;
Catalytic ActivityUDP-alpha-D-galactose + beta-D-galactosyl- (1->4)-beta-N-acetyl-D-glucosaminyl-R = UDP + alpha-D-galactosyl- (1->3)-beta-D-galactosyl-(1->4)-beta-N-acetylglucosaminyl-R.
CofactorName=Mn(2+); Xref=ChEBI:CHEBI:29035; Evidence={ECO:0000250};
Disruption PhenotypeMice are fertile, develop normally and exhibit no overt behavioral abnormalities. However, compared to heterozygous mice they lack expression of the glycosphingolipid isoglobotrihexosylceramide (iGb3) in the dorsal root ganglion. {ECO:0000269|PubMed:17372206}.
DomainThe conserved DXD motif is involved in cofactor binding. The manganese ion interacts with the beta-phosphate group of UDP and may also have a role in catalysis (By similarity). {ECO:0000250}.
FunctionSynthesizes the galactose-alpha(1,3)-galactose group on the glycosphingolipid isoglobotrihexosylceramide or isogloboside 3 (iGb3) by catalyzing the transfer of galactose from UDP-Galactose to its acceptor molecule Gal-beta-1,4-Glc-ceramide. Can also catalyze the addition of galactose to iGb3 itself to form polygalactose structures. Synthesis of iGb3 is the initial step in the formation of the isoglobo-series glycolipid pathway and is the precursor to isogloboside 4 (iGb4) and isoForssman glycolipids. Can glycosylate only lipids and not proteins and is solely responsible for initiating the synthesis of isoglobo-series glycosphingolipids. {ECO:0000269|PubMed:15539565, ECO:0000269|PubMed:16456004}.
PathwayProtein modification; protein glycosylation.
SimilarityBelongs to the glycosyltransferase 6 family. {ECO:0000305}.
Subcellular LocationGolgi apparatus, Golgi stack membrane {ECO:0000305}; Single-pass type II membrane protein {ECO:0000305}. Note=Also found in numerous large vesicles throughout the cytoplasm of the soma. {ECO:0000250}.
Tissue SpecificityThymus and lung. {ECO:0000269|PubMed:16456004}.