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Protein

Ceramide glucosyltransferase

Gene

UGCG

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the first glycosylation step in glycosphingolipid biosynthesis, the transfer of glucose to ceramide. May also serve as a "flippase".1 Publication

Catalytic activityi

UDP-glucose + N-acylsphingosine = UDP + D-glucosyl-N-acylsphingosine.1 Publication

Pathwayi: sphingolipid metabolism

This protein is involved in the pathway sphingolipid metabolism, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway sphingolipid metabolism and in Lipid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei193May play an important role in binding to the inhibitors DEPC and PDMPBy similarity1
Active sitei236Proton acceptorBy similarity1

GO - Molecular functioni

GO - Biological processi

  • epidermis development Source: ProtInc
  • glucosylceramide biosynthetic process Source: ProtInc
  • glycosphingolipid biosynthetic process Source: ProtInc
  • glycosphingolipid metabolic process Source: Reactome
  • positive regulation of protein targeting to mitochondrion Source: ParkinsonsUK-UCL
Complete GO annotation...

Keywords - Molecular functioni

Glycosyltransferase, Transferase

Keywords - Biological processi

Lipid biosynthesis, Lipid metabolism, Sphingolipid metabolism

Enzyme and pathway databases

BioCyciZFISH:HS07494-MONOMER.
BRENDAi2.4.1.80. 2681.
ReactomeiR-HSA-1660662. Glycosphingolipid metabolism.
UniPathwayiUPA00222.

Protein family/group databases

CAZyiGT21. Glycosyltransferase Family 21.
TCDBi4.D.1.4.1. the putative vectorial glycosyl polymerization (vgp) family.

Chemistry databases

SwissLipidsiSLP:000000674.
SLP:000000675.

Names & Taxonomyi

Protein namesi
Recommended name:
Ceramide glucosyltransferase (EC:2.4.1.80)
Alternative name(s):
GLCT-1
Glucosylceramide synthase
Short name:
GCS
UDP-glucose ceramide glucosyltransferase
UDP-glucose:N-acylsphingosine D-glucosyltransferase
Gene namesi
Name:UGCG
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 9

Organism-specific databases

HGNCiHGNC:12524. UGCG.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 10LumenalSequence analysis10
Transmembranei11 – 32HelicalSequence analysisAdd BLAST22
Topological domaini33 – 195CytoplasmicSequence analysisAdd BLAST163
Transmembranei196 – 215HelicalSequence analysisAdd BLAST20
Topological domaini216 – 287LumenalSequence analysisAdd BLAST72
Transmembranei288 – 304HelicalSequence analysisAdd BLAST17
Topological domaini305 – 309CytoplasmicSequence analysis5
Transmembranei310 – 328HelicalSequence analysisAdd BLAST19
Topological domaini329 – 348LumenalSequence analysisAdd BLAST20
Transmembranei349 – 369HelicalSequence analysisAdd BLAST21
Topological domaini370 – 394CytoplasmicSequence analysisAdd BLAST25

GO - Cellular componenti

  • Golgi membrane Source: Reactome
  • integral component of membrane Source: UniProtKB-KW
  • membrane Source: ProtInc
Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi7357.
OpenTargetsiENSG00000148154.
PharmGKBiPA37169.

Chemistry databases

ChEMBLiCHEMBL2063.
DrugBankiDB09039. Eliglustat.
DB00419. Miglustat.
GuidetoPHARMACOLOGYi2528.

Polymorphism and mutation databases

BioMutaiUGCG.
DMDMi2498228.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000591761 – 394Ceramide glucosyltransferaseAdd BLAST394

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei117N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ16739.
MaxQBiQ16739.
PaxDbiQ16739.
PeptideAtlasiQ16739.
PRIDEiQ16739.

PTM databases

iPTMnetiQ16739.
PhosphoSitePlusiQ16739.
SwissPalmiQ16739.

Expressioni

Tissue specificityi

Found in all tissues examined.1 Publication

Gene expression databases

BgeeiENSG00000148154.
CleanExiHS_UGCG.
ExpressionAtlasiQ16739. baseline and differential.
GenevisibleiQ16739. HS.

Organism-specific databases

HPAiHPA024124.

Interactioni

Protein-protein interaction databases

BioGridi113204. 15 interactors.
IntActiQ16739. 2 interactors.
STRINGi9606.ENSP00000363397.

Chemistry databases

BindingDBiQ16739.

Structurei

3D structure databases

ProteinModelPortaliQ16739.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi92D1Curated1
Motifi144D2Curated1
Motifi236D3Curated1
Motifi272 – 276(Q/R)XXRWCurated5

Domaini

The D1, D2, D3, (Q/R)XXRW motif is a critical part of the GCS active site, involved in catalysis and UDP-sugar binding.By similarity

Sequence similaritiesi

Belongs to the glycosyltransferase 2 family.Curated

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG2547. Eukaryota.
COG1215. LUCA.
GeneTreeiENSGT00390000012898.
HOGENOMiHOG000039663.
HOVERGENiHBG003997.
InParanoidiQ16739.
KOiK00720.
OMAiLETFFTM.
OrthoDBiEOG091G0G3M.
PhylomeDBiQ16739.
TreeFamiTF314564.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR025993. Ceramide_glucosylTrfase.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PfamiPF13506. Glyco_transf_21. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 1 hit.

Sequencei

Sequence statusi: Complete.

Q16739-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MALLDLALEG MAVFGFVLFL VLWLMHFMAI IYTRLHLNKK ATDKQPYSKL
60 70 80 90 100
PGVSLLKPLK GVDPNLINNL ETFFELDYPK YEVLLCVQDH DDPAIDVCKK
110 120 130 140 150
LLGKYPNVDA RLFIGGKKVG INPKINNLMP GYEVAKYDLI WICDSGIRVI
160 170 180 190 200
PDTLTDMVNQ MTEKVGLVHG LPYVADRQGF AATLEQVYFG TSHPRYYISA
210 220 230 240 250
NVTGFKCVTG MSCLMRKDVL DQAGGLIAFA QYIAEDYFMA KAIADRGWRF
260 270 280 290 300
AMSTQVAMQN SGSYSISQFQ SRMIRWTKLR INMLPATIIC EPISECFVAS
310 320 330 340 350
LIIGWAAHHV FRWDIMVFFM CHCLAWFIFD YIQLRGVQGG TLCFSKLDYA
360 370 380 390
VAWFIRESMT IYIFLSALWD PTISWRTGRY RLRCGGTAEE ILDV
Length:394
Mass (Da):44,854
Last modified:November 1, 1997 - v1
Checksum:i3B998569F8A96449
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D50840 mRNA. Translation: BAA09451.1.
AK314847 mRNA. Translation: BAG37364.1.
AL442066 Genomic DNA. Translation: CAI15902.1.
CH471105 Genomic DNA. Translation: EAW59091.1.
BC038711 mRNA. Translation: AAH38711.1.
CCDSiCCDS6782.1.
RefSeqiNP_003349.1. NM_003358.2.
UniGeneiHs.304249.
Hs.593014.

Genome annotation databases

EnsembliENST00000374279; ENSP00000363397; ENSG00000148154.
GeneIDi7357.
KEGGihsa:7357.
UCSCiuc004bft.4. human.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D50840 mRNA. Translation: BAA09451.1.
AK314847 mRNA. Translation: BAG37364.1.
AL442066 Genomic DNA. Translation: CAI15902.1.
CH471105 Genomic DNA. Translation: EAW59091.1.
BC038711 mRNA. Translation: AAH38711.1.
CCDSiCCDS6782.1.
RefSeqiNP_003349.1. NM_003358.2.
UniGeneiHs.304249.
Hs.593014.

3D structure databases

ProteinModelPortaliQ16739.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113204. 15 interactors.
IntActiQ16739. 2 interactors.
STRINGi9606.ENSP00000363397.

Chemistry databases

BindingDBiQ16739.
ChEMBLiCHEMBL2063.
DrugBankiDB09039. Eliglustat.
DB00419. Miglustat.
GuidetoPHARMACOLOGYi2528.
SwissLipidsiSLP:000000674.
SLP:000000675.

Protein family/group databases

CAZyiGT21. Glycosyltransferase Family 21.
TCDBi4.D.1.4.1. the putative vectorial glycosyl polymerization (vgp) family.

PTM databases

iPTMnetiQ16739.
PhosphoSitePlusiQ16739.
SwissPalmiQ16739.

Polymorphism and mutation databases

BioMutaiUGCG.
DMDMi2498228.

Proteomic databases

EPDiQ16739.
MaxQBiQ16739.
PaxDbiQ16739.
PeptideAtlasiQ16739.
PRIDEiQ16739.

Protocols and materials databases

DNASUi7357.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000374279; ENSP00000363397; ENSG00000148154.
GeneIDi7357.
KEGGihsa:7357.
UCSCiuc004bft.4. human.

Organism-specific databases

CTDi7357.
DisGeNETi7357.
GeneCardsiUGCG.
HGNCiHGNC:12524. UGCG.
HPAiHPA024124.
MIMi602874. gene.
neXtProtiNX_Q16739.
OpenTargetsiENSG00000148154.
PharmGKBiPA37169.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2547. Eukaryota.
COG1215. LUCA.
GeneTreeiENSGT00390000012898.
HOGENOMiHOG000039663.
HOVERGENiHBG003997.
InParanoidiQ16739.
KOiK00720.
OMAiLETFFTM.
OrthoDBiEOG091G0G3M.
PhylomeDBiQ16739.
TreeFamiTF314564.

Enzyme and pathway databases

UniPathwayiUPA00222.
BioCyciZFISH:HS07494-MONOMER.
BRENDAi2.4.1.80. 2681.
ReactomeiR-HSA-1660662. Glycosphingolipid metabolism.

Miscellaneous databases

ChiTaRSiUGCG. human.
GeneWikiiUGCG.
GenomeRNAii7357.
PROiQ16739.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000148154.
CleanExiHS_UGCG.
ExpressionAtlasiQ16739. baseline and differential.
GenevisibleiQ16739. HS.

Family and domain databases

Gene3Di3.90.550.10. 1 hit.
InterProiIPR025993. Ceramide_glucosylTrfase.
IPR029044. Nucleotide-diphossugar_trans.
[Graphical view]
PfamiPF13506. Glyco_transf_21. 1 hit.
[Graphical view]
SUPFAMiSSF53448. SSF53448. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiCEGT_HUMAN
AccessioniPrimary (citable) accession number: Q16739
Secondary accession number(s): Q5T258
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1997
Last modified: November 2, 2016
This is version 145 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 9
    Human chromosome 9: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.