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Protein

Uroporphyrinogen decarboxylase

Gene

UROD

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

Functioni

Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.

Catalytic activityi

Uroporphyrinogen III = coproporphyrinogen + 4 CO2.

Pathwayi: protoporphyrin-IX biosynthesis

This protein is involved in step 4 of the subpathway that synthesizes coproporphyrinogen-III from 5-aminolevulinate.
Proteins known to be involved in the 4 steps of the subpathway in this organism are:
  1. Delta-aminolevulinic acid dehydratase (ALAD)
  2. Porphobilinogen deaminase (HMBS)
  3. Uroporphyrinogen-III synthase (UROS)
  4. Uroporphyrinogen decarboxylase (UROD)
This subpathway is part of the pathway protoporphyrin-IX biosynthesis, which is itself part of Porphyrin-containing compound metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes coproporphyrinogen-III from 5-aminolevulinate, the pathway protoporphyrin-IX biosynthesis and in Porphyrin-containing compound metabolism.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei55 – 551Substrate
Binding sitei85 – 851Substrate
Binding sitei86 – 861Substrate
Sitei86 – 861Transition state stabilizer
Binding sitei164 – 1641Substrate
Binding sitei219 – 2191Substrate
Binding sitei339 – 3391Substrate

GO - Molecular functioni

  • ferrous iron binding Source: Ensembl
  • uroporphyrinogen decarboxylase activity Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Decarboxylase, Lyase

Keywords - Biological processi

Heme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

BioCyciMetaCyc:HS04993-MONOMER.
BRENDAi4.1.1.37. 2681.
ReactomeiR-HSA-189451. Heme biosynthesis.
UniPathwayiUPA00251; UER00321.

Names & Taxonomyi

Protein namesi
Recommended name:
Uroporphyrinogen decarboxylase (EC:4.1.1.37)
Short name:
UPD
Short name:
URO-D
Gene namesi
Name:UROD
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:12591. UROD.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Involvement in diseasei

Familial porphyria cutanea tarda (FPCT)10 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. Familial porphyria cutanea tarda is an autosomal dominant disorder characterized by light-sensitive dermatitis, with onset in later life. It is associated with the excretion of large amounts of uroporphyrin in the urine. Iron overload is often present in association with varying degrees of liver damage.
See also OMIM:176100
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti25 – 251G → E in FPCT; insoluble protein. 1 Publication
Corresponds to variant rs764268015 [ dbSNP | Ensembl ].
VAR_022567
Natural varianti80 – 801A → S in FPCT; decrease of activity. 2 Publications
Corresponds to variant rs376921379 [ dbSNP | Ensembl ].
VAR_022569
Natural varianti134 – 1341V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications
VAR_009104
Natural varianti142 – 1421R → Q in FPCT. 1 Publication
VAR_010985
Natural varianti144 – 1441R → P in FPCT; decrease of activity. 1 Publication
VAR_022570
Natural varianti156 – 1561G → D in FPCT; decrease of activity. 1 Publication
Corresponds to variant rs762617943 [ dbSNP | Ensembl ].
VAR_022571
Natural varianti161 – 1611L → Q in FPCT. 1 Publication
VAR_010986
Natural varianti165 – 1651M → R in FPCT; activity < 2%. 2 Publications
Corresponds to variant rs121918063 [ dbSNP | Ensembl ].
VAR_007911
Natural varianti167 – 1671E → K in HEP and FPCT; nearly normal activity. 2 Publications
Corresponds to variant rs121918058 [ dbSNP | Ensembl ].
VAR_007714
Natural varianti193 – 1931R → P in FPCT; insoluble protein. 1 Publication
VAR_022572
Natural varianti195 – 1951L → F in FPCT. 1 Publication
Corresponds to variant rs121918064 [ dbSNP | Ensembl ].
VAR_007912
Natural varianti216 – 2161L → Q in FPCT. 1 Publication
VAR_022573
Natural varianti218 – 2181E → K in FPCT; significant decrease of activity. 1 Publication
VAR_022574
Natural varianti219 – 2191S → F in FPCT. 1 Publication
VAR_010987
Natural varianti229 – 2291F → L in FPCT. 1 Publication
VAR_009106
Natural varianti232 – 2321F → L in FPCT; decrease of activity. 1 Publication
VAR_022575
Natural varianti235 – 2351P → S in FPCT. 1 Publication
Corresponds to variant rs141312224 [ dbSNP | Ensembl ].
VAR_010988
Natural varianti253 – 2531L → Q in FPCT; decrease of activity. 2 Publications
Corresponds to variant rs36033115 [ dbSNP | Ensembl ].
VAR_007913
Natural varianti260 – 2601I → T in FPCT; decrease of activity. 1 Publication
VAR_022576
Natural varianti281 – 2811G → E in FPCT and HEP. 3 Publications
Corresponds to variant rs121918057 [ dbSNP | Ensembl ].
VAR_007715
Natural varianti281 – 2811G → V in FPCT. 2 Publications
Corresponds to variant rs121918057 [ dbSNP | Ensembl ].
VAR_007716
Natural varianti282 – 2821L → R in FPCT. 1 Publication
VAR_022577
Natural varianti303 – 3031G → S in FPCT. 1 Publication
VAR_022578
Natural varianti304 – 3041N → K in FPCT. 1 Publication
Corresponds to variant rs121918065 [ dbSNP | Ensembl ].
VAR_007914
Natural varianti318 – 3181G → R in FPCT. 2 Publications
Corresponds to variant rs116233118 [ dbSNP | Ensembl ].
VAR_007915
Natural varianti324 – 3241M → T in FPCT. 1 Publication
VAR_009108
Natural varianti332 – 3321R → H in FPCT. 1 Publication
Corresponds to variant rs121918066 [ dbSNP | Ensembl ].
VAR_007916
Natural varianti334 – 3341I → T in FPCT. 1 Publication
VAR_007917
Hepatoerythropoietic porphyria (HEP)10 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. HEP is a cutaneous porphyria that presents in infancy. It is characterized biochemically by excessive excretion of acetate-substituted porphyrins and accumulation of protoporphyrin in erythrocytes. Uroporphyrinogen decarboxylase levels are very low in erythrocytes and cultured skin fibroblasts.
See also OMIM:176100
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti46 – 461F → L in HEP; mild phenotype; strong decrease of activity. 2 Publications
Corresponds to variant rs769378741 [ dbSNP | Ensembl ].
VAR_022568
Natural varianti62 – 621P → L in HEP. 1 Publication
Corresponds to variant rs121918060 [ dbSNP | Ensembl ].
VAR_009103
Natural varianti80 – 801A → G in HEP. 1 Publication
Corresponds to variant rs776907084 [ dbSNP | Ensembl ].
VAR_007910
Natural varianti134 – 1341V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications
VAR_009104
Natural varianti167 – 1671E → K in HEP and FPCT; nearly normal activity. 2 Publications
Corresponds to variant rs121918058 [ dbSNP | Ensembl ].
VAR_007714
Natural varianti168 – 1681G → R in HEP; relative activity of 65% of wild-type towards uroporphyrinogen III. 1 Publication
VAR_065558
Natural varianti170 – 1701G → D in HEP; relative activity of 17% and 60% of wild-type towards uroporphyrinogen I and III respectively. 1 Publication
VAR_065559
Natural varianti220 – 2201H → P in HEP; mild form. 1 Publication
VAR_009105
Natural varianti281 – 2811G → E in FPCT and HEP. 3 Publications
Corresponds to variant rs121918057 [ dbSNP | Ensembl ].
VAR_007715
Natural varianti292 – 2921R → G in HEP. 1 Publication
Corresponds to variant rs121918059 [ dbSNP | Ensembl ].
VAR_007717
Natural varianti311 – 3111Y → C in HEP. 1 Publication
Corresponds to variant rs121918061 [ dbSNP | Ensembl ].
VAR_009107

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi86 – 861D → E: 5-10% of wild-type activity. 1 Publication
Mutagenesisi86 – 861D → G: Very low activity. Binds substrate with similar geometry as wild-type. 1 Publication
Mutagenesisi86 – 861D → N: No activity. Unable to bind substrate. 1 Publication
Mutagenesisi164 – 1641Y → F: 25-30% of wild-type activity. 1 Publication

Keywords - Diseasei

Disease mutation

Organism-specific databases

MalaCardsiUROD.
MIMi176100. phenotype.
Orphaneti95159. Hepatoerythropoietic porphyria.
101330. Porphyria cutanea tarda.
PharmGKBiPA37221.

Chemistry

ChEMBLiCHEMBL1681619.

Polymorphism and mutation databases

BioMutaiUROD.
DMDMi2507533.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 367367Uroporphyrinogen decarboxylasePRO_0000187569Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei1 – 11N-acetylmethionineCombined sources

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP06132.
MaxQBiP06132.
PaxDbiP06132.
PeptideAtlasiP06132.
PRIDEiP06132.
TopDownProteomicsiP06132.

2D gel databases

OGPiP06132.

PTM databases

iPTMnetiP06132.
PhosphoSiteiP06132.

Expressioni

Gene expression databases

BgeeiENSG00000126088.
CleanExiHS_UROD.
ExpressionAtlasiP06132. baseline and differential.
GenevisibleiP06132. HS.

Organism-specific databases

HPAiHPA027468.
HPA028668.
HPA030350.

Interactioni

Subunit structurei

Homodimer.3 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
FAM84BQ96KN13EBI-2871776,EBI-9057780

Protein-protein interaction databases

BioGridi113235. 19 interactions.
IntActiP06132. 8 interactions.
MINTiMINT-3005024.
STRINGi9606.ENSP00000246337.

Structurei

Secondary structure

1
367
Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Helixi18 – 247Combined sources
Beta strandi38 – 403Combined sources
Helixi44 – 518Combined sources
Helixi55 – 595Combined sources
Helixi62 – 7514Combined sources
Helixi89 – 935Combined sources
Beta strandi99 – 1013Combined sources
Turni102 – 1043Combined sources
Beta strandi105 – 1073Combined sources
Helixi115 – 1206Combined sources
Helixi124 – 1263Combined sources
Helixi127 – 1304Combined sources
Helixi132 – 14514Combined sources
Beta strandi151 – 1566Combined sources
Helixi158 – 16710Combined sources
Helixi175 – 1839Combined sources
Helixi185 – 20824Combined sources
Beta strandi212 – 2187Combined sources
Helixi221 – 2233Combined sources
Helixi226 – 2327Combined sources
Helixi234 – 25017Combined sources
Beta strandi258 – 2625Combined sources
Helixi266 – 2683Combined sources
Helixi269 – 2724Combined sources
Turni273 – 2764Combined sources
Beta strandi278 – 2814Combined sources
Helixi288 – 2958Combined sources
Beta strandi297 – 3059Combined sources
Helixi307 – 3115Combined sources
Helixi314 – 32815Combined sources
Beta strandi330 – 33910Combined sources
Helixi347 – 36519Combined sources

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1JPHX-ray2.10A1-367[»]
1JPIX-ray2.30A1-367[»]
1JPKX-ray2.20A1-367[»]
1R3QX-ray1.70A1-367[»]
1R3RX-ray1.85A1-367[»]
1R3SX-ray1.65A1-367[»]
1R3TX-ray1.70A1-367[»]
1R3VX-ray1.90A1-367[»]
1R3WX-ray1.70A1-367[»]
1R3YX-ray1.75A1-367[»]
1UROX-ray1.80A1-367[»]
2Q6ZX-ray2.00A11-366[»]
2Q71X-ray1.90A11-366[»]
3GVQX-ray2.10A1-367[»]
3GVRX-ray2.20A1-367[»]
3GVVX-ray2.80A1-367[»]
3GVWX-ray2.80A1-367[»]
3GW0X-ray2.00A1-367[»]
3GW3X-ray1.70A1-367[»]
DisProtiDP00308.
ProteinModelPortaliP06132.
SMRiP06132. Positions 10-366.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP06132.

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni37 – 415Substrate binding

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG2872. Eukaryota.
COG0407. LUCA.
GeneTreeiENSGT00390000018302.
HOVERGENiHBG000229.
InParanoidiP06132.
KOiK01599.
OMAiEHVPVWC.
OrthoDBiEOG091G0EUA.
PhylomeDBiP06132.
TreeFamiTF300744.

Family and domain databases

CDDicd00717. URO-D. 1 hit.
HAMAPiMF_00218. URO_D. 1 hit.
InterProiIPR006361. Uroporphyrinogen_deCO2ase_HemE.
IPR000257. Uroporphyrinogen_deCOase.
[Graphical view]
PfamiPF01208. URO-D. 1 hit.
[Graphical view]
TIGRFAMsiTIGR01464. hemE. 1 hit.
PROSITEiPS00906. UROD_1. 1 hit.
PS00907. UROD_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

P06132-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MEANGLGPQG FPELKNDTFL RAAWGEETDY TPVWCMRQAG RYLPEFRETR
60 70 80 90 100
AAQDFFSTCR SPEACCELTL QPLRRFPLDA AIIFSDILVV PQALGMEVTM
110 120 130 140 150
VPGKGPSFPE PLREEQDLER LRDPEVVASE LGYVFQAITL TRQRLAGRVP
160 170 180 190 200
LIGFAGAPWT LMTYMVEGGG SSTMAQAKRW LYQRPQASHQ LLRILTDALV
210 220 230 240 250
PYLVGQVVAG AQALQLFESH AGHLGPQLFN KFALPYIRDV AKQVKARLRE
260 270 280 290 300
AGLAPVPMII FAKDGHFALE ELAQAGYEVV GLDWTVAPKK ARECVGKTVT
310 320 330 340 350
LQGNLDPCAL YASEEEIGQL VKQMLDDFGP HRYIANLGHG LYPDMDPEHV
360
GAFVDAVHKH SRLLRQN
Length:367
Mass (Da):40,787
Last modified:November 1, 1997 - v2
Checksum:i840510B36CFC3856
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti103 – 1031G → S in AAA61258 (PubMed:3015909).Curated
Sequence conflicti103 – 1031G → S in AAB59456 (PubMed:2243121).Curated
Sequence conflicti120 – 1201R → A in AAA61258 (PubMed:3015909).Curated
Sequence conflicti120 – 1201R → A in AAB59456 (PubMed:2243121).Curated
Sequence conflicti212 – 2143Missing in AAB59456 (PubMed:2243121).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti15 – 151K → E.
Corresponds to variant rs11541959 [ dbSNP | Ensembl ].
VAR_060683
Natural varianti25 – 251G → E in FPCT; insoluble protein. 1 Publication
Corresponds to variant rs764268015 [ dbSNP | Ensembl ].
VAR_022567
Natural varianti46 – 461F → L in HEP; mild phenotype; strong decrease of activity. 2 Publications
Corresponds to variant rs769378741 [ dbSNP | Ensembl ].
VAR_022568
Natural varianti62 – 621P → L in HEP. 1 Publication
Corresponds to variant rs121918060 [ dbSNP | Ensembl ].
VAR_009103
Natural varianti77 – 771P → L.3 Publications
Corresponds to variant rs1131147 [ dbSNP | Ensembl ].
VAR_067457
Natural varianti80 – 801A → G in HEP. 1 Publication
Corresponds to variant rs776907084 [ dbSNP | Ensembl ].
VAR_007910
Natural varianti80 – 801A → S in FPCT; decrease of activity. 2 Publications
Corresponds to variant rs376921379 [ dbSNP | Ensembl ].
VAR_022569
Natural varianti106 – 1061P → L.
Corresponds to variant rs11541962 [ dbSNP | Ensembl ].
VAR_060684
Natural varianti113 – 1131R → T.
Corresponds to variant rs11541963 [ dbSNP | Ensembl ].
VAR_060685
Natural varianti134 – 1341V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications
VAR_009104
Natural varianti142 – 1421R → Q in FPCT. 1 Publication
VAR_010985
Natural varianti144 – 1441R → P in FPCT; decrease of activity. 1 Publication
VAR_022570
Natural varianti156 – 1561G → D in FPCT; decrease of activity. 1 Publication
Corresponds to variant rs762617943 [ dbSNP | Ensembl ].
VAR_022571
Natural varianti161 – 1611L → Q in FPCT. 1 Publication
VAR_010986
Natural varianti165 – 1651M → R in FPCT; activity < 2%. 2 Publications
Corresponds to variant rs121918063 [ dbSNP | Ensembl ].
VAR_007911
Natural varianti167 – 1671E → K in HEP and FPCT; nearly normal activity. 2 Publications
Corresponds to variant rs121918058 [ dbSNP | Ensembl ].
VAR_007714
Natural varianti168 – 1681G → R in HEP; relative activity of 65% of wild-type towards uroporphyrinogen III. 1 Publication
VAR_065558
Natural varianti170 – 1701G → D in HEP; relative activity of 17% and 60% of wild-type towards uroporphyrinogen I and III respectively. 1 Publication
VAR_065559
Natural varianti193 – 1931R → P in FPCT; insoluble protein. 1 Publication
VAR_022572
Natural varianti195 – 1951L → F in FPCT. 1 Publication
Corresponds to variant rs121918064 [ dbSNP | Ensembl ].
VAR_007912
Natural varianti216 – 2161L → Q in FPCT. 1 Publication
VAR_022573
Natural varianti218 – 2181E → K in FPCT; significant decrease of activity. 1 Publication
VAR_022574
Natural varianti219 – 2191S → F in FPCT. 1 Publication
VAR_010987
Natural varianti220 – 2201H → P in HEP; mild form. 1 Publication
VAR_009105
Natural varianti229 – 2291F → L in FPCT. 1 Publication
VAR_009106
Natural varianti232 – 2321F → L in FPCT; decrease of activity. 1 Publication
VAR_022575
Natural varianti235 – 2351P → S in FPCT. 1 Publication
Corresponds to variant rs141312224 [ dbSNP | Ensembl ].
VAR_010988
Natural varianti253 – 2531L → Q in FPCT; decrease of activity. 2 Publications
Corresponds to variant rs36033115 [ dbSNP | Ensembl ].
VAR_007913
Natural varianti260 – 2601I → T in FPCT; decrease of activity. 1 Publication
VAR_022576
Natural varianti281 – 2811G → E in FPCT and HEP. 3 Publications
Corresponds to variant rs121918057 [ dbSNP | Ensembl ].
VAR_007715
Natural varianti281 – 2811G → V in FPCT. 2 Publications
Corresponds to variant rs121918057 [ dbSNP | Ensembl ].
VAR_007716
Natural varianti282 – 2821L → R in FPCT. 1 Publication
VAR_022577
Natural varianti292 – 2921R → G in HEP. 1 Publication
Corresponds to variant rs121918059 [ dbSNP | Ensembl ].
VAR_007717
Natural varianti303 – 3031G → S in FPCT. 1 Publication
VAR_022578
Natural varianti303 – 3031G → V.2 Publications
Corresponds to variant rs17849533 [ dbSNP | Ensembl ].
VAR_060686
Natural varianti304 – 3041N → K in FPCT. 1 Publication
Corresponds to variant rs121918065 [ dbSNP | Ensembl ].
VAR_007914
Natural varianti311 – 3111Y → C in HEP. 1 Publication
Corresponds to variant rs121918061 [ dbSNP | Ensembl ].
VAR_009107
Natural varianti318 – 3181G → R in FPCT. 2 Publications
Corresponds to variant rs116233118 [ dbSNP | Ensembl ].
VAR_007915
Natural varianti324 – 3241M → T in FPCT. 1 Publication
VAR_009108
Natural varianti332 – 3321R → H in FPCT. 1 Publication
Corresponds to variant rs121918066 [ dbSNP | Ensembl ].
VAR_007916
Natural varianti334 – 3341I → T in FPCT. 1 Publication
VAR_007917

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M14016 mRNA. Translation: AAA61258.1.
X89267 Genomic DNA. Translation: CAA61540.1.
AF047383 Genomic DNA. Translation: AAC03563.1.
AF104421 mRNA. Translation: AAD04571.1.
AF104422 mRNA. Translation: AAD04572.1.
AF104423 mRNA. Translation: AAD04573.1.
AF104424 mRNA. Translation: AAD04574.1.
AF104425 mRNA. Translation: AAD04575.1.
AF104426 mRNA. Translation: AAD04576.1.
AF104427 mRNA. Translation: AAD04577.1.
AF104428 mRNA. Translation: AAD04578.1.
AF104429 mRNA. Translation: AAD04579.1.
AF104430 mRNA. Translation: AAD04580.1.
AF104431 mRNA. Translation: AAD04581.1.
AF104432 mRNA. Translation: AAD04582.1.
AF104433 mRNA. Translation: AAD04583.1.
AF104434 mRNA. Translation: AAD04584.1.
AF104435 mRNA. Translation: AAD04585.1.
AF104436 mRNA. Translation: AAD04586.1.
AF104437 mRNA. Translation: AAD04587.1.
AF104438 mRNA. Translation: AAD04588.1.
AF104439 mRNA. Translation: AAD04589.1.
AF104440 mRNA. Translation: AAD04590.1.
AY292986 Genomic DNA. Translation: AAP44118.1.
BT006737 mRNA. Translation: AAP35383.1.
CR456976 mRNA. Translation: CAG33257.1.
CR542057 mRNA. Translation: CAG46854.1.
AK291877 mRNA. Translation: BAF84566.1.
AL359473 Genomic DNA. Translation: CAI16440.1.
CH471059 Genomic DNA. Translation: EAX07007.1.
BC001778 mRNA. Translation: AAH01778.1.
U30787 Genomic DNA. Translation: AAC50482.1.
M60891 Genomic DNA. Translation: AAB59456.1.
CCDSiCCDS518.1.
PIRiA24411.
G02786.
RefSeqiNP_000365.3. NM_000374.4.
UniGeneiHs.78601.

Genome annotation databases

EnsembliENST00000246337; ENSP00000246337; ENSG00000126088.
GeneIDi7389.
KEGGihsa:7389.
UCSCiuc001cna.3. human.

Cross-referencesi

Web resourcesi

Wikipedia

Uroporphyrinogen III decarboxylase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M14016 mRNA. Translation: AAA61258.1.
X89267 Genomic DNA. Translation: CAA61540.1.
AF047383 Genomic DNA. Translation: AAC03563.1.
AF104421 mRNA. Translation: AAD04571.1.
AF104422 mRNA. Translation: AAD04572.1.
AF104423 mRNA. Translation: AAD04573.1.
AF104424 mRNA. Translation: AAD04574.1.
AF104425 mRNA. Translation: AAD04575.1.
AF104426 mRNA. Translation: AAD04576.1.
AF104427 mRNA. Translation: AAD04577.1.
AF104428 mRNA. Translation: AAD04578.1.
AF104429 mRNA. Translation: AAD04579.1.
AF104430 mRNA. Translation: AAD04580.1.
AF104431 mRNA. Translation: AAD04581.1.
AF104432 mRNA. Translation: AAD04582.1.
AF104433 mRNA. Translation: AAD04583.1.
AF104434 mRNA. Translation: AAD04584.1.
AF104435 mRNA. Translation: AAD04585.1.
AF104436 mRNA. Translation: AAD04586.1.
AF104437 mRNA. Translation: AAD04587.1.
AF104438 mRNA. Translation: AAD04588.1.
AF104439 mRNA. Translation: AAD04589.1.
AF104440 mRNA. Translation: AAD04590.1.
AY292986 Genomic DNA. Translation: AAP44118.1.
BT006737 mRNA. Translation: AAP35383.1.
CR456976 mRNA. Translation: CAG33257.1.
CR542057 mRNA. Translation: CAG46854.1.
AK291877 mRNA. Translation: BAF84566.1.
AL359473 Genomic DNA. Translation: CAI16440.1.
CH471059 Genomic DNA. Translation: EAX07007.1.
BC001778 mRNA. Translation: AAH01778.1.
U30787 Genomic DNA. Translation: AAC50482.1.
M60891 Genomic DNA. Translation: AAB59456.1.
CCDSiCCDS518.1.
PIRiA24411.
G02786.
RefSeqiNP_000365.3. NM_000374.4.
UniGeneiHs.78601.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
1JPHX-ray2.10A1-367[»]
1JPIX-ray2.30A1-367[»]
1JPKX-ray2.20A1-367[»]
1R3QX-ray1.70A1-367[»]
1R3RX-ray1.85A1-367[»]
1R3SX-ray1.65A1-367[»]
1R3TX-ray1.70A1-367[»]
1R3VX-ray1.90A1-367[»]
1R3WX-ray1.70A1-367[»]
1R3YX-ray1.75A1-367[»]
1UROX-ray1.80A1-367[»]
2Q6ZX-ray2.00A11-366[»]
2Q71X-ray1.90A11-366[»]
3GVQX-ray2.10A1-367[»]
3GVRX-ray2.20A1-367[»]
3GVVX-ray2.80A1-367[»]
3GVWX-ray2.80A1-367[»]
3GW0X-ray2.00A1-367[»]
3GW3X-ray1.70A1-367[»]
DisProtiDP00308.
ProteinModelPortaliP06132.
SMRiP06132. Positions 10-366.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113235. 19 interactions.
IntActiP06132. 8 interactions.
MINTiMINT-3005024.
STRINGi9606.ENSP00000246337.

Chemistry

ChEMBLiCHEMBL1681619.

PTM databases

iPTMnetiP06132.
PhosphoSiteiP06132.

Polymorphism and mutation databases

BioMutaiUROD.
DMDMi2507533.

2D gel databases

OGPiP06132.

Proteomic databases

EPDiP06132.
MaxQBiP06132.
PaxDbiP06132.
PeptideAtlasiP06132.
PRIDEiP06132.
TopDownProteomicsiP06132.

Protocols and materials databases

DNASUi7389.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000246337; ENSP00000246337; ENSG00000126088.
GeneIDi7389.
KEGGihsa:7389.
UCSCiuc001cna.3. human.

Organism-specific databases

CTDi7389.
GeneCardsiUROD.
GeneReviewsiUROD.
HGNCiHGNC:12591. UROD.
HPAiHPA027468.
HPA028668.
HPA030350.
MalaCardsiUROD.
MIMi176100. phenotype.
613521. gene.
neXtProtiNX_P06132.
Orphaneti95159. Hepatoerythropoietic porphyria.
101330. Porphyria cutanea tarda.
PharmGKBiPA37221.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2872. Eukaryota.
COG0407. LUCA.
GeneTreeiENSGT00390000018302.
HOVERGENiHBG000229.
InParanoidiP06132.
KOiK01599.
OMAiEHVPVWC.
OrthoDBiEOG091G0EUA.
PhylomeDBiP06132.
TreeFamiTF300744.

Enzyme and pathway databases

UniPathwayiUPA00251; UER00321.
BioCyciMetaCyc:HS04993-MONOMER.
BRENDAi4.1.1.37. 2681.
ReactomeiR-HSA-189451. Heme biosynthesis.

Miscellaneous databases

ChiTaRSiUROD. human.
EvolutionaryTraceiP06132.
GeneWikiiUroporphyrinogen_III_decarboxylase.
GenomeRNAii7389.
PROiP06132.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000126088.
CleanExiHS_UROD.
ExpressionAtlasiP06132. baseline and differential.
GenevisibleiP06132. HS.

Family and domain databases

CDDicd00717. URO-D. 1 hit.
HAMAPiMF_00218. URO_D. 1 hit.
InterProiIPR006361. Uroporphyrinogen_deCO2ase_HemE.
IPR000257. Uroporphyrinogen_deCOase.
[Graphical view]
PfamiPF01208. URO-D. 1 hit.
[Graphical view]
TIGRFAMsiTIGR01464. hemE. 1 hit.
PROSITEiPS00906. UROD_1. 1 hit.
PS00907. UROD_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiDCUP_HUMAN
AccessioniPrimary (citable) accession number: P06132
Secondary accession number(s): A8K762
, Q16863, Q16883, Q53YB8, Q53ZP6, Q6IB28, Q9BUZ0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: November 1, 1997
Last modified: September 7, 2016
This is version 192 of the entry and version 2 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. 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.