Q8NBZ7 (UXS1_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

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 names

Name:	UXS1
ORF Names:	UNQ2538/PRO6079

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	420 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Catalyzes the NAD-dependent decarboxylation of UDP-glucuronic acid to UDP-xylose. Necessary for the biosynthesis of the core tetrasaccharide in glycosaminoglycan biosynthesis.
Catalytic activity	UDP-D-glucuronate = UDP-D-xylose + CO2.
Cofactor	NAD.
Pathway	Nucleotide-sugar biosynthesis; UDP-alpha-D-xylose biosynthesis; UDP-alpha-D-xylose from UDP-alpha-D-glucuronate: step 1/1.
Subunit structure	Interacts with AKT1 By similarity.
Subcellular location	Golgi apparatus › Golgi stack membrane; Single-pass type II membrane protein By similarity.
Sequence similarities	Belongs to the sugar epimerase family. UDP-glucuronic acid decarboxylase subfamily.

Ontologies

Keywords
Cellular component	Golgi apparatus Membrane
Coding sequence diversity	Alternative splicing
Domain	Signal-anchor Transmembrane Transmembrane helix
Ligand	NAD
Molecular function	Decarboxylase Lyase
PTM	Glycoprotein
Technical term	3D-structure Complete proteome Reference proteome
Gene Ontology (GO)
Biological process	cellular metabolic process Inferred from electronic annotation. Source: InterPro
Cellular component	Golgi cisterna membrane Inferred from electronic annotation. Source: UniProtKB-SubCell integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW
Molecular function	UDP-glucuronate decarboxylase activity Inferred from electronic annotation. Source: EC coenzyme binding Inferred from electronic annotation. Source: InterPro nucleotide binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Alternative products

This entry describes 3 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: Q8NBZ7-1)

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.

Isoform 2 (identifier: Q8NBZ7-2)

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

Isoform 3 (identifier: Q8NBZ7-3)

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

Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 420

420

UDP-glucuronic acid decarboxylase 1

PRO_0000183269

Regions

Topological domain

1 – 19

19

Cytoplasmic Potential

Transmembrane

20 – 40

21

Helical; Signal-anchor for type II membrane protein; Potential

Topological domain

41 – 420

380

Lumenal Potential

Nucleotide binding

119 – 144

26

NAD

Nucleotide binding

231 – 235

5

NAD

Nucleotide binding

273 – 277

5

Substrate

Nucleotide binding

290 – 297

8

Substrate

Nucleotide binding

357 – 361

5

Substrate

Sites

Binding site

228

1

Substrate

Binding site

260

1

Substrate

Binding site

272

1

NAD

Amino acid modifications

Glycosylation

316

1

N-linked (GlcNAc...) Potential

Natural variations

Alternative sequence

1 – 168

168

Missing in isoform 3.

VSP_016756

Alternative sequence

40

1

M → MSFLLN in isoform 2.

VSP_016757

Secondary structure

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420

Helix Strand Turn

Details...

Beta strand

90 – 94

5

Turn

95 – 97

3

Helix

99 – 110

12

Beta strand

114 – 119

6

Helix

126 – 128

3

Helix

130 – 132

3

Beta strand

138 – 142

5

Beta strand

154 – 158

5

Helix

165 – 168

4

Helix

172 – 193

22

Beta strand

196 – 202

7

Helix

203 – 206

4

Beta strand

210 – 214

5

Beta strand

224 – 226

3

Helix

229 – 249

21

Beta strand

253 – 258

6

Helix

273 – 283

11

Beta strand

287 – 293

7

Beta strand

296 – 298

3

Helix

302 – 313

12

Beta strand

321 – 324

4

Beta strand

328 – 330

3

Helix

331 – 342

12

Beta strand

348 – 351

4

Helix

366 – 372

7

Helix

380 – 398

19

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified October 1, 2002. Version 1. Checksum: FD7DC0E1B1D02157	FASTA	420	47,577
	10 20 30 40 50 60 MVSKALLRLV SAVNRRRMKL LLGIALLAYV ASVWGNFVNM RSIQENGELK IESKIEEMVE 70 80 90 100 110 120 PLREKIRDLE KSFTQKYPPV KFLSEKDRKR ILITGGAGFV GSHLTDKLMM DGHEVTVVDN 130 140 150 160 170 180 FFTGRKRNVE HWIGHENFEL INHDVVEPLY IEVDQIYHLA SPASPPNYMY NPIKTLKTNT 190 200 210 220 230 240 IGTLNMLGLA KRVGARLLLA STSEVYGDPE VHPQSEDYWG HVNPIGPRAC YDEGKRVAET 250 260 270 280 290 300 MCYAYMKQEG VEVRVARIFN TFGPRMHMND GRVVSNFILQ ALQGEPLTVY GSGSQTRAFQ 310 320 330 340 350 360 YVSDLVNGLV ALMNSNVSSP VNLGNPEEHT ILEFAQLIKN LVGSGSEIQF LSEAQDDPQK 370 380 390 400 410 420 RKPDIKKAKL MLGWEPVVPL EEGLNKAIHY FRKELEYQAN NQYIPKPKPA RIKKGRTRHS
	Isoform 2 [UniParc]. Checksum: 155C013E0C58C159	FASTA	425	48,151
	10 20 30 40 50 60 MVSKALLRLV SAVNRRRMKL LLGIALLAYV ASVWGNFVNM SFLLNRSIQE NGELKIESKI 70 80 90 100 110 120 EEMVEPLREK IRDLEKSFTQ KYPPVKFLSE KDRKRILITG GAGFVGSHLT DKLMMDGHEV 130 140 150 160 170 180 TVVDNFFTGR KRNVEHWIGH ENFELINHDV VEPLYIEVDQ IYHLASPASP PNYMYNPIKT 190 200 210 220 230 240 LKTNTIGTLN MLGLAKRVGA RLLLASTSEV YGDPEVHPQS EDYWGHVNPI GPRACYDEGK 250 260 270 280 290 300 RVAETMCYAY MKQEGVEVRV ARIFNTFGPR MHMNDGRVVS NFILQALQGE PLTVYGSGSQ 310 320 330 340 350 360 TRAFQYVSDL VNGLVALMNS NVSSPVNLGN PEEHTILEFA QLIKNLVGSG SEIQFLSEAQ 370 380 390 400 410 420 DDPQKRKPDI KKAKLMLGWE PVVPLEEGLN KAIHYFRKEL EYQANNQYIP KPKPARIKKG RTRHS
	Isoform 3 [UniParc]. Checksum: A4A1687AED8AF62D	FASTA	252	28,275
	10 20 30 40 50 60 MYNPIKTLKT NTIGTLNMLG LAKRVGARLL LASTSEVYGD PEVHPQSEDY WGHVNPIGPR 70 80 90 100 110 120 ACYDEGKRVA ETMCYAYMKQ EGVEVRVARI FNTFGPRMHM NDGRVVSNFI LQALQGEPLT 130 140 150 160 170 180 VYGSGSQTRA FQYVSDLVNG LVALMNSNVS SPVNLGNPEE HTILEFAQLI KNLVGSGSEI 190 200 210 220 230 240 QFLSEAQDDP QKRKPDIKKA KLMLGWEPVV PLEEGLNKAI HYFRKELEYQ ANNQYIPKPK 250 PARIKKGRTR HS

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"The SQV-1 UDP-glucuronic acid decarboxylase and the SQV-7 nucleotide-sugar transporter may act in the Golgi apparatus to affect Caenorhabditis elegans vulval morphogenesis and embryonic development." Hwang H.-Y., Horvitz H.R. Proc. Natl. Acad. Sci. U.S.A. 99:14218-14223(2002) [PubMed: 12391314] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], ALTERNATIVE SPLICING.
[2]	"The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment." Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J., Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P., Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E. , Heldens S., Huang A., Kim H.S., Klimowski L., Jin Y., Johnson S., Lee J., Lewis L., Liao D., Mark M.R., Robbie E., Sanchez C., Schoenfeld J., Seshagiri S., Simmons L., Singh J., Smith V., Stinson J., Vagts A., Vandlen R.L., Watanabe C., Wieand D., Woods K., Xie M.-H., Yansura D.G., Yi S., Yu G., Yuan J., Zhang M., Zhang Z., Goddard A.D., Wood W.I., Godowski P.J., Gray A.M. Genome Res. 13:2265-2270(2003) [PubMed: 12975309] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
[3]	"Complete sequencing and characterization of 21,243 full-length human cDNAs." Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S. Nat. Genet. 36:40-45(2004) [PubMed: 14702039] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1; 2 AND 3). Tissue: Placenta.
[4]	"Generation and annotation of the DNA sequences of human chromosomes 2 and 4." Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H. , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K. Nature 434:724-731(2005) [PubMed: 15815621] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1). Tissue: Placenta.
[6]	"Crystal structure of human UDP-glucoronic acid decarboxylase." Structural genomics consortium (SGC) Submitted (OCT-2005) to the PDB data bank Cited for: X-RAY CRYSTALLOGRAPHY (1.21 ANGSTROMS) OF 85-402 IN COMPLEX WITH NAD AND UDP.
+	Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ

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.

IPI

IPI00410544.
IPI00657807.
IPI00658111.

RefSeq

NP_079352.2. NM_025076.3.

UniGene

Hs.469561.

3D structure databases

PDBe
RCSB PDB
PDBj

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
2B69	X-ray	1.21	A	85-402	[»]

ProteinModelPortal

Q8NBZ7.

SMR

Q8NBZ7. Positions 88-399.

ModBase

Search...

Protein-protein interaction databases

IntAct

Q8NBZ7. 1 interaction.

STRING

Q8NBZ7.

PTM databases

PhosphoSite

Q8NBZ7.

Polymorphism databases

DMDM

74730150.

Proteomic databases

PRIDE

Q8NBZ7.

Protocols and materials databases

StructuralBiologyKnowledgebase

Search...

Genome annotation databases

Ensembl

ENST00000409501; ENSP00000387019; ENSG00000115652.

GeneID

80146.

KEGG

hsa:80146.

UCSC

uc002tdl.1. human.
uc002tdm.1. human.

Organism-specific databases

CTD

80146.

GeneCards

GC02M106709.

H-InvDB

HIX0002349.
HIX0030285.

HGNC

HGNC:17729. UXS1.

HPA

HPA008825.

MIM

609749. gene.

neXtProt

NX_Q8NBZ7.

PharmGKB

PA38465.

GenAtlas

Search...

Phylogenomic databases

eggNOG

prNOG11975.

HOVERGEN

HBG094144.

OMA

EMVEPLR.

PhylomeDB

Q8NBZ7.

Gene expression databases

ArrayExpress

Q8NBZ7.

Bgee

Q8NBZ7.

CleanEx

HS_UXS1.

Genevestigator

Q8NBZ7.

GermOnline

ENSG00000115652. Homo sapiens.

Family and domain databases

InterPro

IPR001509. Epimerase_deHydtase.
IPR016040. NAD(P)-bd_dom.
IPR021761. UXS1_N.
[Graphical view]

Gene3D

G3DSA:3.40.50.720. NAD(P)-bd. 1 hit.

KO

K08678.

Pfam

PF01370. Epimerase. 1 hit.
PF11803. UXS1_N. 1 hit.
[Graphical view]

ProtoNet

Search...

Other

NextBio

70422.

SOURCE

Search...

Entry information

Entry name

UXS1_HUMAN

Accession

Primary (citable) accession number: Q8NBZ7
Secondary accession number(s): Q8NBX3, Q9H5C2

Entry history

Integrated into UniProtKB/Swiss-Prot:	December 20, 2005
Last sequence update:	October 1, 2002
Last modified:	January 25, 2012
This is version 84 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.