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Protein

UDP-glucuronic acid decarboxylase 1

Gene

UXS1

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

Functioni

Catalyzes the NAD-dependent decarboxylation of UDP-glucuronic acid to UDP-xylose. Necessary for the biosynthesis of the core tetrasaccharide in glycosaminoglycan biosynthesis.

Catalytic activityi

UDP-D-glucuronate = UDP-D-xylose + CO2.

Cofactori

Pathwayi: UDP-alpha-D-xylose biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes UDP-alpha-D-xylose from UDP-alpha-D-glucuronate.
Proteins known to be involved in this subpathway in this organism are:
  1. UDP-glucuronic acid decarboxylase 1 (UXS1)
This subpathway is part of the pathway UDP-alpha-D-xylose biosynthesis, which is itself part of Nucleotide-sugar biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes UDP-alpha-D-xylose from UDP-alpha-D-glucuronate, the pathway UDP-alpha-D-xylose biosynthesis and in Nucleotide-sugar biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei228Substrate1
Active sitei231Proton acceptorBy similarity1
Binding sitei260Substrate1
Binding sitei272NAD1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi119 – 144NAD1 PublicationAdd BLAST26
Nucleotide bindingi231 – 235NAD1 Publication5
Nucleotide bindingi273 – 277Substrate5
Nucleotide bindingi290 – 297Substrate8
Nucleotide bindingi357 – 361Substrate5

GO - Molecular functioni

  • NAD+ binding Source: UniProtKB
  • protein homodimerization activity Source: UniProtKB
  • UDP-glucuronate decarboxylase activity Source: UniProtKB

GO - Biological processi

  • protein tetramerization Source: UniProtKB
  • UDP-D-xylose biosynthetic process Source: UniProtKB-UniPathway
Complete GO annotation...

Keywords - Molecular functioni

Decarboxylase, Lyase

Keywords - Ligandi

NAD

Enzyme and pathway databases

BioCyciZFISH:HS03922-MONOMER.
UniPathwayiUPA00796; UER00771.

Names & Taxonomyi

Protein namesi
Recommended name:
UDP-glucuronic acid decarboxylase 1 (EC:4.1.1.35)
Alternative name(s):
UDP-glucuronate decarboxylase 1
Short name:
UGD
Short name:
UXS-1
Gene namesi
Name:UXS1
ORF Names:UNQ2538/PRO6079
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

HGNCiHGNC:17729. UXS1.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini1 – 19CytoplasmicSequence analysisAdd BLAST19
Transmembranei20 – 40Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
Topological domaini41 – 420LumenalSequence analysisAdd BLAST380

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi80146.
OpenTargetsiENSG00000115652.
PharmGKBiPA38465.

Polymorphism and mutation databases

BioMutaiUXS1.
DMDMi74730150.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001832691 – 420UDP-glucuronic acid decarboxylase 1Add BLAST420

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei94PhosphothreonineCombined sources1
Glycosylationi316N-linked (GlcNAc...)Sequence analysis1

Keywords - PTMi

Glycoprotein, Phosphoprotein

Proteomic databases

EPDiQ8NBZ7.
MaxQBiQ8NBZ7.
PaxDbiQ8NBZ7.
PeptideAtlasiQ8NBZ7.
PRIDEiQ8NBZ7.

PTM databases

iPTMnetiQ8NBZ7.
PhosphoSitePlusiQ8NBZ7.

Expressioni

Gene expression databases

BgeeiENSG00000115652.
CleanExiHS_UXS1.
ExpressionAtlasiQ8NBZ7. baseline and differential.
GenevisibleiQ8NBZ7. HS.

Organism-specific databases

HPAiHPA008825.

Interactioni

Subunit structurei

Interacts with AKT1.By similarity

GO - Molecular functioni

  • protein homodimerization activity Source: UniProtKB

Protein-protein interaction databases

BioGridi123139. 36 interactors.
IntActiQ8NBZ7. 3 interactors.
STRINGi9606.ENSP00000387019.

Structurei

Secondary structure

1420
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi90 – 94Combined sources5
Turni95 – 97Combined sources3
Helixi99 – 110Combined sources12
Beta strandi114 – 119Combined sources6
Beta strandi122 – 124Combined sources3
Helixi126 – 128Combined sources3
Helixi130 – 132Combined sources3
Beta strandi138 – 142Combined sources5
Beta strandi154 – 158Combined sources5
Helixi165 – 168Combined sources4
Helixi172 – 193Combined sources22
Beta strandi196 – 202Combined sources7
Helixi203 – 206Combined sources4
Beta strandi210 – 214Combined sources5
Beta strandi224 – 226Combined sources3
Helixi229 – 249Combined sources21
Beta strandi253 – 258Combined sources6
Helixi273 – 283Combined sources11
Beta strandi287 – 293Combined sources7
Beta strandi296 – 298Combined sources3
Helixi302 – 313Combined sources12
Beta strandi321 – 324Combined sources4
Beta strandi328 – 330Combined sources3
Helixi331 – 342Combined sources12
Beta strandi348 – 351Combined sources4
Helixi366 – 372Combined sources7
Helixi380 – 398Combined sources19

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2B69X-ray1.21A85-402[»]
4GLLX-ray2.50A/B85-420[»]
4LK3X-ray2.64A/B/C/D/E/F85-420[»]
4M55X-ray2.86A/B/C/D/E/F85-420[»]
ProteinModelPortaliQ8NBZ7.
SMRiQ8NBZ7.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ8NBZ7.

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1429. Eukaryota.
COG0451. LUCA.
GeneTreeiENSGT00530000063128.
HOGENOMiHOG000168004.
HOVERGENiHBG094144.
InParanoidiQ8NBZ7.
KOiK08678.
OMAiPKVKYLN.
OrthoDBiEOG091G0BV7.
PhylomeDBiQ8NBZ7.
TreeFamiTF105736.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR016040. NAD(P)-bd_dom.
IPR021761. UXS1_N.
[Graphical view]
PfamiPF16363. GDP_Man_Dehyd. 1 hit.
PF11803. UXS1_N. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8NBZ7-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MVSKALLRLV SAVNRRRMKL LLGIALLAYV ASVWGNFVNM RSIQENGELK
60 70 80 90 100
IESKIEEMVE PLREKIRDLE KSFTQKYPPV KFLSEKDRKR ILITGGAGFV
110 120 130 140 150
GSHLTDKLMM DGHEVTVVDN FFTGRKRNVE HWIGHENFEL INHDVVEPLY
160 170 180 190 200
IEVDQIYHLA SPASPPNYMY NPIKTLKTNT IGTLNMLGLA KRVGARLLLA
210 220 230 240 250
STSEVYGDPE VHPQSEDYWG HVNPIGPRAC YDEGKRVAET MCYAYMKQEG
260 270 280 290 300
VEVRVARIFN TFGPRMHMND GRVVSNFILQ ALQGEPLTVY GSGSQTRAFQ
310 320 330 340 350
YVSDLVNGLV ALMNSNVSSP VNLGNPEEHT ILEFAQLIKN LVGSGSEIQF
360 370 380 390 400
LSEAQDDPQK RKPDIKKAKL MLGWEPVVPL EEGLNKAIHY FRKELEYQAN
410 420
NQYIPKPKPA RIKKGRTRHS
Length:420
Mass (Da):47,577
Last modified:October 1, 2002 - v1
Checksum:iFD7DC0E1B1D02157
GO
Isoform 2 (identifier: Q8NBZ7-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     40-40: M → MSFLLN

Show »
Length:425
Mass (Da):48,151
Checksum:i155C013E0C58C159
GO
Isoform 3 (identifier: Q8NBZ7-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-168: Missing.

Show »
Length:252
Mass (Da):28,275
Checksum:iA4A1687AED8AF62D
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0167561 – 168Missing in isoform 3. 1 PublicationAdd BLAST168
Alternative sequenceiVSP_01675740M → MSFLLN in isoform 2. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY147934 mRNA. Translation: AAN39844.1.
AY358541 mRNA. Translation: AAQ88905.1.
AK027244 mRNA. Translation: BAB15705.1.
AK075120 mRNA. Translation: BAC11415.1.
AK075170 mRNA. Translation: BAC11448.1.
AC018878 Genomic DNA. Translation: AAY15085.1.
BC009819 mRNA. Translation: AAH09819.2.
CCDSiCCDS46378.1. [Q8NBZ7-1]
CCDS58720.1. [Q8NBZ7-3]
CCDS58721.1. [Q8NBZ7-2]
RefSeqiNP_001240804.1. NM_001253875.1. [Q8NBZ7-2]
NP_001240805.1. NM_001253876.1. [Q8NBZ7-3]
NP_079352.2. NM_025076.4. [Q8NBZ7-1]
XP_016860503.1. XM_017005014.1. [Q8NBZ7-3]
UniGeneiHs.469561.
Hs.743389.

Genome annotation databases

EnsembliENST00000283148; ENSP00000283148; ENSG00000115652. [Q8NBZ7-2]
ENST00000409032; ENSP00000387096; ENSG00000115652. [Q8NBZ7-3]
ENST00000409501; ENSP00000387019; ENSG00000115652. [Q8NBZ7-1]
GeneIDi80146.
KEGGihsa:80146.
UCSCiuc002tdl.4. human. [Q8NBZ7-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY147934 mRNA. Translation: AAN39844.1.
AY358541 mRNA. Translation: AAQ88905.1.
AK027244 mRNA. Translation: BAB15705.1.
AK075120 mRNA. Translation: BAC11415.1.
AK075170 mRNA. Translation: BAC11448.1.
AC018878 Genomic DNA. Translation: AAY15085.1.
BC009819 mRNA. Translation: AAH09819.2.
CCDSiCCDS46378.1. [Q8NBZ7-1]
CCDS58720.1. [Q8NBZ7-3]
CCDS58721.1. [Q8NBZ7-2]
RefSeqiNP_001240804.1. NM_001253875.1. [Q8NBZ7-2]
NP_001240805.1. NM_001253876.1. [Q8NBZ7-3]
NP_079352.2. NM_025076.4. [Q8NBZ7-1]
XP_016860503.1. XM_017005014.1. [Q8NBZ7-3]
UniGeneiHs.469561.
Hs.743389.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2B69X-ray1.21A85-402[»]
4GLLX-ray2.50A/B85-420[»]
4LK3X-ray2.64A/B/C/D/E/F85-420[»]
4M55X-ray2.86A/B/C/D/E/F85-420[»]
ProteinModelPortaliQ8NBZ7.
SMRiQ8NBZ7.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi123139. 36 interactors.
IntActiQ8NBZ7. 3 interactors.
STRINGi9606.ENSP00000387019.

PTM databases

iPTMnetiQ8NBZ7.
PhosphoSitePlusiQ8NBZ7.

Polymorphism and mutation databases

BioMutaiUXS1.
DMDMi74730150.

Proteomic databases

EPDiQ8NBZ7.
MaxQBiQ8NBZ7.
PaxDbiQ8NBZ7.
PeptideAtlasiQ8NBZ7.
PRIDEiQ8NBZ7.

Protocols and materials databases

DNASUi80146.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000283148; ENSP00000283148; ENSG00000115652. [Q8NBZ7-2]
ENST00000409032; ENSP00000387096; ENSG00000115652. [Q8NBZ7-3]
ENST00000409501; ENSP00000387019; ENSG00000115652. [Q8NBZ7-1]
GeneIDi80146.
KEGGihsa:80146.
UCSCiuc002tdl.4. human. [Q8NBZ7-1]

Organism-specific databases

CTDi80146.
DisGeNETi80146.
GeneCardsiUXS1.
H-InvDBHIX0030285.
HGNCiHGNC:17729. UXS1.
HPAiHPA008825.
MIMi609749. gene.
neXtProtiNX_Q8NBZ7.
OpenTargetsiENSG00000115652.
PharmGKBiPA38465.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1429. Eukaryota.
COG0451. LUCA.
GeneTreeiENSGT00530000063128.
HOGENOMiHOG000168004.
HOVERGENiHBG094144.
InParanoidiQ8NBZ7.
KOiK08678.
OMAiPKVKYLN.
OrthoDBiEOG091G0BV7.
PhylomeDBiQ8NBZ7.
TreeFamiTF105736.

Enzyme and pathway databases

UniPathwayiUPA00796; UER00771.
BioCyciZFISH:HS03922-MONOMER.

Miscellaneous databases

ChiTaRSiUXS1. human.
EvolutionaryTraceiQ8NBZ7.
GeneWikiiUXS1.
GenomeRNAii80146.
PROiQ8NBZ7.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000115652.
CleanExiHS_UXS1.
ExpressionAtlasiQ8NBZ7. baseline and differential.
GenevisibleiQ8NBZ7. HS.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR016040. NAD(P)-bd_dom.
IPR021761. UXS1_N.
[Graphical view]
PfamiPF16363. GDP_Man_Dehyd. 1 hit.
PF11803. UXS1_N. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiUXS1_HUMAN
AccessioniPrimary (citable) accession number: Q8NBZ7
Secondary accession number(s): Q8NBX3, Q9H5C2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 20, 2005
Last sequence update: October 1, 2002
Last modified: November 2, 2016
This is version 127 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 2
    Human chromosome 2: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. 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.