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Protein

Ceramide glucosyltransferase

Gene

UGCG

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionGlycosyltransferase, Transferase
Biological processLipid biosynthesis, Lipid metabolism, Sphingolipid metabolism

Enzyme and pathway databases

BioCyciMetaCyc:HS07494-MONOMER
BRENDAi2.4.1.80 2681
ReactomeiR-HSA-1660662 Glycosphingolipid metabolism
SIGNORiQ16739
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

EuPathDBiHostDB:ENSG00000148154.9
HGNCiHGNC:12524 UGCG
MIMi602874 gene
neXtProtiNX_Q16739

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi7357
OpenTargetsiENSG00000148154
PharmGKBiPA37169

Chemistry databases

ChEMBLiCHEMBL2063
DrugBankiDB09039 Eliglustat
DB05715 Genz-112638
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, 16 interactors
IntActiQ16739, 2 interactors
MINTiQ16739
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
InterProiView protein in InterPro
IPR025993 Ceramide_glucosylTrfase
IPR029044 Nucleotide-diphossugar_trans
PfamiView protein in Pfam
PF13506 Glyco_transf_21, 1 hit
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 No translation available.
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

Similar proteinsi

Entry informationi

Entry nameiCEGT_HUMAN
AccessioniPrimary (citable) accession number: Q16739
Secondary accession number(s): Q5T258
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 1, 1997
Last modified: May 23, 2018
This is version 153 of the entry and version 1 of the sequence. See 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

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