UniProtKB - P06132 (DCUP_HUMAN)
Protein
Uroporphyrinogen decarboxylase
Gene
UROD
Organism
Homo sapiens (Human)
Status
Functioni
Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
Catalytic activityi
- EC:4.1.1.37
: protoporphyrin-IX biosynthesis Pathwayi
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:
- Delta-aminolevulinic acid dehydratase, Porphobilinogen synthase (ALAD), Delta-aminolevulinic acid dehydratase (ALAD), Delta-aminolevulinic acid dehydratase, Delta-aminolevulinic acid dehydratase (ALAD), Delta-aminolevulinic acid dehydratase
- Hydroxymethylbilane synthase (PBGD), Hydroxymethylbilane synthase (HMBS), Porphobilinogen deaminase (HMBS), Hydroxymethylbilane synthase (HMBS), Hydroxymethylbilane synthase (HMBS), Hydroxymethylbilane synthase (HMBS)
- Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Uroporphyrinogen-III synthase (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS), Hydroxymethylbilane hydrolyase [cyclizing] (UROS)
- Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD)
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 key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 55 | Substrate | 1 | |
Binding sitei | 85 | Substrate | 1 | |
Binding sitei | 86 | Substrate | 1 | |
Sitei | 86 | Transition state stabilizer | 1 | |
Binding sitei | 164 | Substrate | 1 | |
Binding sitei | 219 | Substrate | 1 | |
Binding sitei | 339 | Substrate | 1 |
GO - Molecular functioni
- uroporphyrinogen decarboxylase activity Source: UniProtKB
GO - Biological processi
- heme biosynthetic process Source: GO_Central
- protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
Keywordsi
Molecular function | Decarboxylase, Lyase |
Biological process | Heme biosynthesis, Porphyrin biosynthesis |
Enzyme and pathway databases
BioCyci | MetaCyc:HS04993-MONOMER |
BRENDAi | 4.1.1.37, 2681 |
PathwayCommonsi | P06132 |
Reactomei | R-HSA-189451, Heme biosynthesis |
UniPathwayi | UPA00251;UER00321 |
Names & Taxonomyi
Protein namesi | |
Gene namesi | Name:UROD |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:12591, UROD |
MIMi | 613521, gene |
neXtProti | NX_P06132 |
VEuPathDBi | HostDB:ENSG00000126088.12 |
Subcellular locationi
Cytoplasm and Cytosol
Cytosol
- cytosol Source: HPA
Nucleus
- nucleoplasm Source: HPA
Keywords - Cellular componenti
CytoplasmPathology & Biotechi
Involvement in diseasei
Familial porphyria cutanea tarda (FPCT)10 Publications
The disease is caused by variants 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.
Related information in OMIMFeature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_022567 | 25 | G → E in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs764268015Ensembl. | 1 | |
Natural variantiVAR_022569 | 80 | A → S in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs376921379Ensembl. | 1 | |
Natural variantiVAR_009104 | 134 | V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications | 1 | |
Natural variantiVAR_010985 | 142 | R → Q in FPCT. 1 PublicationCorresponds to variant dbSNP:rs1182234844Ensembl. | 1 | |
Natural variantiVAR_022570 | 144 | R → P in FPCT; decrease of activity. 1 Publication | 1 | |
Natural variantiVAR_022571 | 156 | G → D in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs762617943Ensembl. | 1 | |
Natural variantiVAR_010986 | 161 | L → Q in FPCT. 1 Publication | 1 | |
Natural variantiVAR_007911 | 165 | M → R in FPCT; activity < 2%. 2 PublicationsCorresponds to variant dbSNP:rs121918063EnsemblClinVar. | 1 | |
Natural variantiVAR_007714 | 167 | E → K in HEP and FPCT; nearly normal activity. 2 PublicationsCorresponds to variant dbSNP:rs121918058EnsemblClinVar. | 1 | |
Natural variantiVAR_022572 | 193 | R → P in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs143823335EnsemblClinVar. | 1 | |
Natural variantiVAR_007912 | 195 | L → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918064EnsemblClinVar. | 1 | |
Natural variantiVAR_022573 | 216 | L → Q in FPCT. 1 Publication | 1 | |
Natural variantiVAR_022574 | 218 | E → K in FPCT; significant decrease of activity. 1 Publication | 1 | |
Natural variantiVAR_010987 | 219 | S → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs982293378Ensembl. | 1 | |
Natural variantiVAR_009106 | 229 | F → L in FPCT. 1 Publication | 1 | |
Natural variantiVAR_022575 | 232 | F → L in FPCT; decrease of activity. 1 Publication | 1 | |
Natural variantiVAR_010988 | 235 | P → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs141312224Ensembl. | 1 | |
Natural variantiVAR_007913 | 253 | L → Q in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs36033115EnsemblClinVar. | 1 | |
Natural variantiVAR_022576 | 260 | I → T in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs1483459837Ensembl. | 1 | |
Natural variantiVAR_007715 | 281 | G → E in FPCT and HEP. 3 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar. | 1 | |
Natural variantiVAR_007716 | 281 | G → V in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar. | 1 | |
Natural variantiVAR_022577 | 282 | L → R in FPCT. 1 Publication | 1 | |
Natural variantiVAR_022578 | 303 | G → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs964670864Ensembl. | 1 | |
Natural variantiVAR_007914 | 304 | N → K in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918065EnsemblClinVar. | 1 | |
Natural variantiVAR_007915 | 318 | G → R in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs116233118Ensembl. | 1 | |
Natural variantiVAR_009108 | 324 | M → T in FPCT. 1 PublicationCorresponds to variant dbSNP:rs763746230Ensembl. | 1 | |
Natural variantiVAR_007916 | 332 | R → H in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918066EnsemblClinVar. | 1 | |
Natural variantiVAR_007917 | 334 | I → T in FPCT. 1 Publication | 1 |
Hepatoerythropoietic porphyria (HEP)10 Publications
The disease is caused by variants 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.
Related information in OMIMFeature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_022568 | 46 | F → L in HEP; mild phenotype; strong decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs769378741Ensembl. | 1 | |
Natural variantiVAR_009103 | 62 | P → L in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918060EnsemblClinVar. | 1 | |
Natural variantiVAR_007910 | 80 | A → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs776907084Ensembl. | 1 | |
Natural variantiVAR_009104 | 134 | V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications | 1 | |
Natural variantiVAR_007714 | 167 | E → K in HEP and FPCT; nearly normal activity. 2 PublicationsCorresponds to variant dbSNP:rs121918058EnsemblClinVar. | 1 | |
Natural variantiVAR_065558 | 168 | G → R in HEP; relative activity of 65% of wild-type towards uroporphyrinogen III. 1 Publication | 1 | |
Natural variantiVAR_065559 | 170 | G → D in HEP; relative activity of 17% and 60% of wild-type towards uroporphyrinogen I and III respectively. 1 Publication | 1 | |
Natural variantiVAR_009105 | 220 | H → P in HEP; mild form. 1 Publication | 1 | |
Natural variantiVAR_007715 | 281 | G → E in FPCT and HEP. 3 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar. | 1 | |
Natural variantiVAR_007717 | 292 | R → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918059EnsemblClinVar. | 1 | |
Natural variantiVAR_009107 | 311 | Y → C in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918061EnsemblClinVar. | 1 |
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 86 | D → E: 5-10% of wild-type activity. 1 Publication | 1 | |
Mutagenesisi | 86 | D → G: Very low activity. Binds substrate with similar geometry as wild-type. 1 Publication | 1 | |
Mutagenesisi | 86 | D → N: No activity. Unable to bind substrate. 1 Publication | 1 | |
Mutagenesisi | 164 | Y → F: 25-30% of wild-type activity. 1 Publication | 1 |
Keywords - Diseasei
Disease variantOrganism-specific databases
DisGeNETi | 7389 |
GeneReviewsi | UROD |
MalaCardsi | UROD |
MIMi | 176100, phenotype |
OpenTargetsi | ENSG00000126088 |
Orphaneti | 443062, Familial porphyria cutanea tarda 95159, Hepatoerythropoietic porphyria |
PharmGKBi | PA37221 |
Miscellaneous databases
Pharosi | P06132, Tbio |
Chemistry databases
ChEMBLi | CHEMBL1681619 |
DrugBanki | DB03727, Coproporphyrin I DB04461, Coproporphyrinogen III |
Genetic variation databases
BioMutai | UROD |
DMDMi | 2507533 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000187569 | 1 – 367 | Uroporphyrinogen decarboxylaseAdd BLAST | 367 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 1 | N-acetylmethionineCombined sources | 1 |
Keywords - PTMi
AcetylationProteomic databases
EPDi | P06132 |
jPOSTi | P06132 |
MassIVEi | P06132 |
MaxQBi | P06132 |
PaxDbi | P06132 |
PeptideAtlasi | P06132 |
PRIDEi | P06132 |
ProteomicsDBi | 51870 |
TopDownProteomicsi | P06132 |
2D gel databases
OGPi | P06132 |
PTM databases
iPTMneti | P06132 |
MetOSitei | P06132 |
PhosphoSitePlusi | P06132 |
Expressioni
Gene expression databases
Bgeei | ENSG00000126088, Expressed in liver and 242 other tissues |
ExpressionAtlasi | P06132, baseline and differential |
Genevisiblei | P06132, HS |
Organism-specific databases
HPAi | ENSG00000126088, Low tissue specificity |
Interactioni
Subunit structurei
Homodimer.
3 PublicationsBinary interactionsi
Hide detailsP06132
With | #Exp. | IntAct |
---|---|---|
LRATD2 [Q96KN1] | 7 | EBI-2871776,EBI-9057780 |
PHKA2 [P46019] | 3 | EBI-2871776,EBI-1642846 |
PILRA [C9JJ79] | 5 | EBI-2871776,EBI-12117156 |
TSSK3 [Q96PN8] | 3 | EBI-2871776,EBI-3918381 |
Protein-protein interaction databases
BioGRIDi | 113235, 26 interactors |
IntActi | P06132, 14 interactors |
MINTi | P06132 |
STRINGi | 9606.ENSP00000246337 |
Miscellaneous databases
RNActi | P06132, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
SMRi | P06132 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P06132 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 37 – 41 | Substrate binding | 5 |
Sequence similaritiesi
Belongs to the uroporphyrinogen decarboxylase family.Curated
Phylogenomic databases
eggNOGi | KOG2872, Eukaryota |
GeneTreei | ENSGT00390000018302 |
HOGENOMi | CLU_040933_0_0_1 |
InParanoidi | P06132 |
OMAi | GSSKDFR |
OrthoDBi | 1114675at2759 |
PhylomeDBi | P06132 |
TreeFami | TF300744 |
Family and domain databases
CDDi | cd00717, URO-D, 1 hit |
DisProti | DP00308 |
Gene3Di | 3.20.20.210, 1 hit |
HAMAPi | MF_00218, URO_D, 1 hit |
InterProi | View protein in InterPro IPR038071, UROD/MetE-like_sf IPR006361, Uroporphyrinogen_deCO2ase_HemE IPR000257, Uroporphyrinogen_deCOase |
Pfami | View protein in Pfam PF01208, URO-D, 1 hit |
SUPFAMi | SSF51726, SSF51726, 1 hit |
TIGRFAMsi | TIGR01464, hemE, 1 hit |
PROSITEi | View protein in PROSITE PS00906, UROD_1, 1 hit PS00907, UROD_2, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
This entry has 1 described isoform and 13 potential isoforms that are computationally mapped.Show allAlign All
P06132-1 [UniParc]FASTAAdd to basket
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
Computationally mapped potential isoform sequencesi
There are 13 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketA0A1B0GVZ4 | A0A1B0GVZ4_HUMAN | Uroporphyrinogen decarboxylase | UROD | 321 | Annotation score: | ||
Q5T446 | Q5T446_HUMAN | Uroporphyrinogen decarboxylase | UROD | 275 | Annotation score: | ||
A0A494C085 | A0A494C085_HUMAN | Uroporphyrinogen decarboxylase | UROD | 346 | Annotation score: | ||
A0A494C0Q8 | A0A494C0Q8_HUMAN | Uroporphyrinogen decarboxylase | UROD | 332 | Annotation score: | ||
A0A1B0GVN9 | A0A1B0GVN9_HUMAN | Uroporphyrinogen decarboxylase | UROD | 298 | Annotation score: | ||
A0A494C0S8 | A0A494C0S8_HUMAN | Uroporphyrinogen decarboxylase | UROD | 247 | Annotation score: | ||
A0A494BZY8 | A0A494BZY8_HUMAN | Uroporphyrinogen decarboxylase | UROD | 286 | Annotation score: | ||
A0A494C1D8 | A0A494C1D8_HUMAN | Uroporphyrinogen decarboxylase | UROD | 93 | Annotation score: | ||
A0A494C007 | A0A494C007_HUMAN | Uroporphyrinogen decarboxylase | UROD | 135 | Annotation score: | ||
A0A494C1T3 | A0A494C1T3_HUMAN | Uroporphyrinogen decarboxylase | UROD | 100 | Annotation score: | ||
There are more potential isoformsShow all |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 103 | G → S in AAA61258 (PubMed:3015909).Curated | 1 | |
Sequence conflicti | 103 | G → S in AAB59456 (PubMed:2243121).Curated | 1 | |
Sequence conflicti | 120 | R → A in AAA61258 (PubMed:3015909).Curated | 1 | |
Sequence conflicti | 120 | R → A in AAB59456 (PubMed:2243121).Curated | 1 | |
Sequence conflicti | 212 – 214 | Missing in AAB59456 (PubMed:2243121).Curated | 3 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_060683 | 15 | K → E. Corresponds to variant dbSNP:rs11541959Ensembl. | 1 | |
Natural variantiVAR_022567 | 25 | G → E in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs764268015Ensembl. | 1 | |
Natural variantiVAR_022568 | 46 | F → L in HEP; mild phenotype; strong decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs769378741Ensembl. | 1 | |
Natural variantiVAR_009103 | 62 | P → L in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918060EnsemblClinVar. | 1 | |
Natural variantiVAR_067457 | 77 | P → L3 PublicationsCorresponds to variant dbSNP:rs1131147Ensembl. | 1 | |
Natural variantiVAR_007910 | 80 | A → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs776907084Ensembl. | 1 | |
Natural variantiVAR_022569 | 80 | A → S in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs376921379Ensembl. | 1 | |
Natural variantiVAR_060684 | 106 | P → L. Corresponds to variant dbSNP:rs11541962Ensembl. | 1 | |
Natural variantiVAR_060685 | 113 | R → T. Corresponds to variant dbSNP:rs11541963Ensembl. | 1 | |
Natural variantiVAR_009104 | 134 | V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications | 1 | |
Natural variantiVAR_010985 | 142 | R → Q in FPCT. 1 PublicationCorresponds to variant dbSNP:rs1182234844Ensembl. | 1 | |
Natural variantiVAR_022570 | 144 | R → P in FPCT; decrease of activity. 1 Publication | 1 | |
Natural variantiVAR_022571 | 156 | G → D in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs762617943Ensembl. | 1 | |
Natural variantiVAR_010986 | 161 | L → Q in FPCT. 1 Publication | 1 | |
Natural variantiVAR_007911 | 165 | M → R in FPCT; activity < 2%. 2 PublicationsCorresponds to variant dbSNP:rs121918063EnsemblClinVar. | 1 | |
Natural variantiVAR_007714 | 167 | E → K in HEP and FPCT; nearly normal activity. 2 PublicationsCorresponds to variant dbSNP:rs121918058EnsemblClinVar. | 1 | |
Natural variantiVAR_065558 | 168 | G → R in HEP; relative activity of 65% of wild-type towards uroporphyrinogen III. 1 Publication | 1 | |
Natural variantiVAR_065559 | 170 | G → D in HEP; relative activity of 17% and 60% of wild-type towards uroporphyrinogen I and III respectively. 1 Publication | 1 | |
Natural variantiVAR_022572 | 193 | R → P in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs143823335EnsemblClinVar. | 1 | |
Natural variantiVAR_007912 | 195 | L → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918064EnsemblClinVar. | 1 | |
Natural variantiVAR_022573 | 216 | L → Q in FPCT. 1 Publication | 1 | |
Natural variantiVAR_022574 | 218 | E → K in FPCT; significant decrease of activity. 1 Publication | 1 | |
Natural variantiVAR_010987 | 219 | S → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs982293378Ensembl. | 1 | |
Natural variantiVAR_009105 | 220 | H → P in HEP; mild form. 1 Publication | 1 | |
Natural variantiVAR_009106 | 229 | F → L in FPCT. 1 Publication | 1 | |
Natural variantiVAR_022575 | 232 | F → L in FPCT; decrease of activity. 1 Publication | 1 | |
Natural variantiVAR_010988 | 235 | P → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs141312224Ensembl. | 1 | |
Natural variantiVAR_007913 | 253 | L → Q in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs36033115EnsemblClinVar. | 1 | |
Natural variantiVAR_022576 | 260 | I → T in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs1483459837Ensembl. | 1 | |
Natural variantiVAR_007715 | 281 | G → E in FPCT and HEP. 3 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar. | 1 | |
Natural variantiVAR_007716 | 281 | G → V in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar. | 1 | |
Natural variantiVAR_022577 | 282 | L → R in FPCT. 1 Publication | 1 | |
Natural variantiVAR_007717 | 292 | R → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918059EnsemblClinVar. | 1 | |
Natural variantiVAR_022578 | 303 | G → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs964670864Ensembl. | 1 | |
Natural variantiVAR_060686 | 303 | G → V2 PublicationsCorresponds to variant dbSNP:rs17849533Ensembl. | 1 | |
Natural variantiVAR_007914 | 304 | N → K in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918065EnsemblClinVar. | 1 | |
Natural variantiVAR_009107 | 311 | Y → C in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918061EnsemblClinVar. | 1 | |
Natural variantiVAR_007915 | 318 | G → R in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs116233118Ensembl. | 1 | |
Natural variantiVAR_009108 | 324 | M → T in FPCT. 1 PublicationCorresponds to variant dbSNP:rs763746230Ensembl. | 1 | |
Natural variantiVAR_007916 | 332 | R → H in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918066EnsemblClinVar. | 1 | |
Natural variantiVAR_007917 | 334 | I → T in FPCT. 1 Publication | 1 |
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 No translation available. CH471059 Genomic DNA Translation: EAX07007.1 BC001778 mRNA Translation: AAH01778.1 U30787 Genomic DNA Translation: AAC50482.1 M60891 Genomic DNA Translation: AAB59456.1 |
CCDSi | CCDS518.1 |
PIRi | A24411 G02786 |
RefSeqi | NP_000365.3, NM_000374.4 |
Genome annotation databases
Ensembli | ENST00000246337; ENSP00000246337; ENSG00000126088 |
GeneIDi | 7389 |
KEGGi | hsa:7389 |
UCSCi | uc001cna.3, human |
Similar proteinsi
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 No translation available. CH471059 Genomic DNA Translation: EAX07007.1 BC001778 mRNA Translation: AAH01778.1 U30787 Genomic DNA Translation: AAC50482.1 M60891 Genomic DNA Translation: AAB59456.1 |
CCDSi | CCDS518.1 |
PIRi | A24411 G02786 |
RefSeqi | NP_000365.3, NM_000374.4 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
1JPH | X-ray | 2.10 | A | 1-367 | [»] | |
1JPI | X-ray | 2.30 | A | 1-367 | [»] | |
1JPK | X-ray | 2.20 | A | 1-367 | [»] | |
1R3Q | X-ray | 1.70 | A | 1-367 | [»] | |
1R3R | X-ray | 1.85 | A | 1-367 | [»] | |
1R3S | X-ray | 1.65 | A | 1-367 | [»] | |
1R3T | X-ray | 1.70 | A | 1-367 | [»] | |
1R3V | X-ray | 1.90 | A | 1-367 | [»] | |
1R3W | X-ray | 1.70 | A | 1-367 | [»] | |
1R3Y | X-ray | 1.75 | A | 1-367 | [»] | |
1URO | X-ray | 1.80 | A | 1-367 | [»] | |
2Q6Z | X-ray | 2.00 | A | 11-366 | [»] | |
2Q71 | X-ray | 1.90 | A | 11-366 | [»] | |
3GVQ | X-ray | 2.10 | A | 1-367 | [»] | |
3GVR | X-ray | 2.20 | A | 1-367 | [»] | |
3GVV | X-ray | 2.80 | A | 1-367 | [»] | |
3GVW | X-ray | 2.80 | A | 1-367 | [»] | |
3GW0 | X-ray | 2.00 | A | 1-367 | [»] | |
3GW3 | X-ray | 1.70 | A | 1-367 | [»] | |
SMRi | P06132 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 113235, 26 interactors |
IntActi | P06132, 14 interactors |
MINTi | P06132 |
STRINGi | 9606.ENSP00000246337 |
Chemistry databases
ChEMBLi | CHEMBL1681619 |
DrugBanki | DB03727, Coproporphyrin I DB04461, Coproporphyrinogen III |
PTM databases
iPTMneti | P06132 |
MetOSitei | P06132 |
PhosphoSitePlusi | P06132 |
Genetic variation databases
BioMutai | UROD |
DMDMi | 2507533 |
2D gel databases
OGPi | P06132 |
Proteomic databases
EPDi | P06132 |
jPOSTi | P06132 |
MassIVEi | P06132 |
MaxQBi | P06132 |
PaxDbi | P06132 |
PeptideAtlasi | P06132 |
PRIDEi | P06132 |
ProteomicsDBi | 51870 |
TopDownProteomicsi | P06132 |
Protocols and materials databases
Antibodypediai | 18538, 238 antibodies |
DNASUi | 7389 |
Genome annotation databases
Ensembli | ENST00000246337; ENSP00000246337; ENSG00000126088 |
GeneIDi | 7389 |
KEGGi | hsa:7389 |
UCSCi | uc001cna.3, human |
Organism-specific databases
CTDi | 7389 |
DisGeNETi | 7389 |
GeneCardsi | UROD |
GeneReviewsi | UROD |
HGNCi | HGNC:12591, UROD |
HPAi | ENSG00000126088, Low tissue specificity |
MalaCardsi | UROD |
MIMi | 176100, phenotype 613521, gene |
neXtProti | NX_P06132 |
OpenTargetsi | ENSG00000126088 |
Orphaneti | 443062, Familial porphyria cutanea tarda 95159, Hepatoerythropoietic porphyria |
PharmGKBi | PA37221 |
VEuPathDBi | HostDB:ENSG00000126088.12 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2872, Eukaryota |
GeneTreei | ENSGT00390000018302 |
HOGENOMi | CLU_040933_0_0_1 |
InParanoidi | P06132 |
OMAi | GSSKDFR |
OrthoDBi | 1114675at2759 |
PhylomeDBi | P06132 |
TreeFami | TF300744 |
Enzyme and pathway databases
UniPathwayi | UPA00251;UER00321 |
BioCyci | MetaCyc:HS04993-MONOMER |
BRENDAi | 4.1.1.37, 2681 |
PathwayCommonsi | P06132 |
Reactomei | R-HSA-189451, Heme biosynthesis |
Miscellaneous databases
BioGRID-ORCSi | 7389, 497 hits in 995 CRISPR screens |
ChiTaRSi | UROD, human |
EvolutionaryTracei | P06132 |
GeneWikii | Uroporphyrinogen_III_decarboxylase |
GenomeRNAii | 7389 |
Pharosi | P06132, Tbio |
PROi | PR:P06132 |
RNActi | P06132, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000126088, Expressed in liver and 242 other tissues |
ExpressionAtlasi | P06132, baseline and differential |
Genevisiblei | P06132, HS |
Family and domain databases
CDDi | cd00717, URO-D, 1 hit |
DisProti | DP00308 |
Gene3Di | 3.20.20.210, 1 hit |
HAMAPi | MF_00218, URO_D, 1 hit |
InterProi | View protein in InterPro IPR038071, UROD/MetE-like_sf IPR006361, Uroporphyrinogen_deCO2ase_HemE IPR000257, Uroporphyrinogen_deCOase |
Pfami | View protein in Pfam PF01208, URO-D, 1 hit |
SUPFAMi | SSF51726, SSF51726, 1 hit |
TIGRFAMsi | TIGR01464, hemE, 1 hit |
PROSITEi | View protein in PROSITE PS00906, UROD_1, 1 hit PS00907, UROD_2, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | DCUP_HUMAN | |
Accessioni | P06132Primary (citable) accession number: P06132 Secondary accession number(s): A8K762 Q9BUZ0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 1, 1988 |
Last sequence update: | November 1, 1997 | |
Last modified: | April 7, 2021 | |
This is version 223 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | 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. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 1
Human chromosome 1: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families