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Protein

UDP-glucuronic acid decarboxylase 1

Gene

UXS1

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

Keywordsi

Molecular functionDecarboxylase, Lyase
LigandNAD

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000115652.14
HGNCiHGNC:17729 UXS1
MIMi609749 gene
neXtProtiNX_Q8NBZ7

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 – 19CytoplasmicSequence analysisAdd BLAST19
Transmembranei20 – 40Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST21
Topological domaini41 – 420LumenalSequence analysisAdd BLAST380

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 residuei1N-acetylmethionine1 Publication1
Modified residuei94PhosphothreonineCombined sources1
Glycosylationi316N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Acetylation, Glycoprotein, Phosphoprotein

Proteomic databases

EPDiQ8NBZ7
MaxQBiQ8NBZ7
PaxDbiQ8NBZ7
PeptideAtlasiQ8NBZ7
PRIDEiQ8NBZ7
ProteomicsDBi72836
72837 [Q8NBZ7-2]
72838 [Q8NBZ7-3]

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, 42 interactors
IntActiQ8NBZ7, 3 interactors
MINTiQ8NBZ7
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

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
OMAiHWLGHEN
OrthoDBiEOG091G0BV7
PhylomeDBiQ8NBZ7
TreeFamiTF105736

Family and domain databases

InterProiView protein in InterPro
IPR016040 NAD(P)-bd_dom
IPR036291 NAD(P)-bd_dom_sf
IPR021761 UXS1_N
PfamiView protein in Pfam
PF16363 GDP_Man_Dehyd, 1 hit
PF11803 UXS1_N, 1 hit
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

Similar proteinsi

Entry informationi

Entry nameiUXS1_HUMAN
AccessioniPrimary (citable) accession number: Q8NBZ7
Secondary accession number(s): Q8NBX3, Q9H5C2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 20, 2005
Last sequence update: October 1, 2002
Last modified: July 18, 2018
This is version 139 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

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

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