ID   A4GAT_PONPY             Reviewed;         218 AA.
AC   Q9N289;
DT   25-NOV-2002, integrated into UniProtKB/Swiss-Prot.
DT   01-OCT-2000, sequence version 1.
DT   25-MAY-2022, entry version 81.
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   Pongo pygmaeus (Bornean orangutan).
OC   Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC   Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC   Pongo.
OX   NCBI_TaxID=9600;
RN   [1]
RP   NUCLEOTIDE SEQUENCE [GENOMIC DNA].
RC   STRAIN=Isolate oran-Po17;
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; AB041421; BAA94506.1; -; Genomic_DNA.
DR   AlphaFoldDB; Q9N289; -.
DR   CAZy; GT32; Glycosyltransferase Family 32.
DR   GO; GO:0000139; C:Golgi membrane; IEA:UniProtKB-SubCell.
DR   GO; GO:0050512; F:lactosylceramide 4-alpha-galactosyltransferase activity; IEA:UniProtKB-EC.
DR   GO; GO:0006629; P:lipid metabolic process; IEA:UniProtKB-KW.
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; Transferase.
FT   CHAIN           <1..218
FT                   /note="Lactosylceramide 4-alpha-galactosyltransferase"
FT                   /id="PRO_0000080581"
FT   MOTIF           57..59
FT                   /note="DXD motif"
FT                   /evidence="ECO:0000250"
FT   NON_TER         1
SQ   SEQUENCE   218 AA;  25622 MW;  57958B41C318ABC5 CRC64;
     FPNVQMLPLD LRELFRDTPL ADWYTAVQGR WEPYLLPVLS DASRIALMWK FGGIYLDTDF
     IVLKNLRNLT NVLGTQSRYV LNGAFLAFQR RHEFMALCMR DFVDHYNGWI WGHQGPQLLT
     RVFKKWCSIR SLAESRACRG VTTLPPEAFY PIPWQDWKKY FEDISPEELP RLLNATYAVH
     VWNKKSQGTR FEATSRALLA QLHARYCPTT HEAMKMYL