ID   A4GAT_GORGO             Reviewed;         327 AA.
AC   Q9N290;
DT   25-NOV-2002, integrated into UniProtKB/Swiss-Prot.
DT   01-OCT-2000, sequence version 1.
DT   29-SEP-2021, entry version 72.
DE   RecName: Full=Lactosylceramide 4-alpha-galactosyltransferase;
DE            EC=2.4.1.228 {ECO:0000250|UniProtKB:Q9NPC4};
DE   AltName: Full=Alpha-1,4-N-acetylglucosaminyltransferase;
DE   AltName: Full=Alpha-1,4-galactosyltransferase;
DE   AltName: Full=Globotriaosylceramide synthase;
DE            Short=Gb3 synthase;
DE   AltName: Full=UDP-galactose:beta-D-galactosyl-beta1-R 4-alpha-D-galactosyltransferase;
DE   Flags: Fragment;
GN   Name=A4GALT; Synonyms=A14GALT, A4GALT1;
OS   Gorilla gorilla gorilla (Western lowland gorilla).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC   Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC   Gorilla.
OX   NCBI_TaxID=9595;
RN   [1]
RP   NUCLEOTIDE SEQUENCE [GENOMIC DNA].
RX   PubMed=15014171; DOI=10.1093/molbev/msh100;
RA   Kitano T., Liu Y.-H., Ueda S., Saitou N.;
RT   "Human-specific amino acid changes found in 103 protein-coding genes.";
RL   Mol. Biol. Evol. 21:936-944(2004).
CC   -!- FUNCTION: Catalyzes the transfer of galactose from UDP-alpha-D-
CC       galactose to lactosylceramide/beta-D-galactosyl-(1->4)-beta-D-glucosyl-
CC       (1<->1)-ceramide(d18:1(4E)) to produce globotriaosylceramide/globoside
CC       Gb3Cer (d18:1(4E)). Also able to transfer galactose to
CC       galactosylceramide/beta-D-Gal-(1<->1')-Cer. Globoside Gb3Cer is a
CC       glycosphingolipid of the globo serie, one of the major types of neutral
CC       root structures of glycosphingolipids, that constitute a significant
CC       portion of mammalian cell membranes. {ECO:0000250|UniProtKB:Q9NPC4}.
CC   -!- CATALYTIC ACTIVITY:
CC       Reaction=a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) + UDP-
CC         alpha-D-galactose = globoside Gb3Cer (d18:1(4E)) + H(+) + UDP;
CC         Xref=Rhea:RHEA:11924, ChEBI:CHEBI:15378, ChEBI:CHEBI:17950,
CC         ChEBI:CHEBI:18313, ChEBI:CHEBI:58223, ChEBI:CHEBI:66914;
CC         EC=2.4.1.228; Evidence={ECO:0000250|UniProtKB:Q9NPC4};
CC       PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:11925;
CC         Evidence={ECO:0000250|UniProtKB:Q9NPC4};
CC   -!- CATALYTIC ACTIVITY:
CC       Reaction=beta-D-Gal-(1<->1')-Cer + UDP-alpha-D-galactose = alpha-D-Gal-
CC         (1->4)-beta-D-Gal-(1<->1')-Cer + H(+) + UDP; Xref=Rhea:RHEA:60044,
CC         ChEBI:CHEBI:15378, ChEBI:CHEBI:58223, ChEBI:CHEBI:66914,
CC         ChEBI:CHEBI:143593, ChEBI:CHEBI:143594;
CC         Evidence={ECO:0000250|UniProtKB:Q9NPC4};
CC       PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:60045;
CC         Evidence={ECO:0000250|UniProtKB:Q9NPC4};
CC   -!- PATHWAY: Glycolipid biosynthesis. {ECO:0000250|UniProtKB:Q9NPC4}.
CC   -!- SUBCELLULAR LOCATION: Golgi apparatus membrane {ECO:0000305}; Single-
CC       pass type II membrane protein {ECO:0000305}.
CC   -!- DOMAIN: The conserved DXD motif is involved in enzyme activity.
CC       {ECO:0000250}.
CC   -!- SIMILARITY: Belongs to the glycosyltransferase 32 family.
CC       {ECO:0000305}.
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DR   EMBL; AB041420; BAA94505.1; -; Genomic_DNA.
DR   STRING; 9593.ENSGGOP00000009135; -.
DR   eggNOG; KOG1928; Eukaryota.
DR   InParanoid; Q9N290; -.
DR   Proteomes; UP000001519; Unplaced.
DR   GO; GO:0000139; C:Golgi membrane; IEA:UniProtKB-SubCell.
DR   GO; GO:0008378; F:galactosyltransferase activity; ISS:UniProtKB.
DR   GO; GO:0016758; F:hexosyltransferase activity; IBA:GO_Central.
DR   GO; GO:0050512; F:lactosylceramide 4-alpha-galactosyltransferase activity; IEA:UniProtKB-EC.
DR   GO; GO:0006688; P:glycosphingolipid biosynthetic process; IBA:GO_Central.
DR   InterPro; IPR007652; A1-4-GlycosylTfrase_dom.
DR   InterPro; IPR007577; GlycoTrfase_DXD_sugar-bd_CS.
DR   InterPro; IPR029044; Nucleotide-diphossugar_trans.
DR   Pfam; PF04572; Gb3_synth; 1.
DR   Pfam; PF04488; Gly_transf_sug; 1.
DR   SUPFAM; SSF53448; SSF53448; 1.
PE   3: Inferred from homology;
KW   Glycosyltransferase; Golgi apparatus; Lipid biosynthesis; Lipid metabolism;
KW   Membrane; Reference proteome; Transferase.
FT   CHAIN           <1..327
FT                   /note="Lactosylceramide 4-alpha-galactosyltransferase"
FT                   /id="PRO_0000080577"
FT   MOTIF           166..168
FT                   /note="DXD motif"
FT                   /evidence="ECO:0000250"
FT   NON_TER         1
SQ   SEQUENCE   327 AA;  37394 MW;  ED9A9A37A19F66B8 CRC64;
     GFKFTFFVSI MIYWHVVGEP KEKGQLYNLP AEIPCPTLAP PTPPSHGPAP GNIFFLETSD
     RTNPNFLFMC SVESAARTHP ESHVLVLMKG LPGGNASLPR HLGISLLSCF PNVQMLPLDL
     RELFRDTPLA DWYAAVQGRW EPYLLPVLSD ASRIALMWKF GGIYLDTDFI VLKNLRNLTN
     VLGTQSRYVL NGAFLAFERX HEFMALCMXD FVDHYNGWIW GHQGPQLLTR VFKKWCSIRS
     LAESRACRGV TTLPPEAFYP IPWQDWKKYF EDINPEELPR LFSATYAVHV WNKKSQGTRF
     EATSRALLAQ LHARYCPTTH EAMKMYL