UROD

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:

Delta-aminolevulinic acid dehydratase (ALAD)
Porphobilinogen deaminase (HMBS)
Uroporphyrinogen-III synthase (UROS)
Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (UROD), Uroporphyrinogen decarboxylase (hemE)

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 key	Position(s)	DescriptionActions	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
Inferred from direct assayⁱ
- 18004775

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

heme biosynthetic process
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway

View the complete GO annotation on QuickGO ...

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
Reactomeⁱ	R-HSA-189451 Heme biosynthesis
UniPathwayⁱ	UPA00251;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
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 1

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000126088.12
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:12591 UROD
MIMⁱ	613521 gene
neXtProtⁱ	NX_P06132

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.

Feature key	Position(s)	DescriptionActions	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_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_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_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 Publication	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 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.

Feature key	Position(s)	DescriptionActions	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_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_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	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 mutation

Organism-specific databases

DisGeNET More...DisGeNETⁱ	7389
GeneReviewsⁱ	UROD
MalaCards human disease database More...MalaCardsⁱ	UROD
MIMⁱ	176100 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000126088
Orphanetⁱ	443062 Familial porphyria cutanea tarda 95159 Hepatoerythropoietic porphyria
PharmGKBⁱ	PA37221

Chemistry databases

ChEMBLⁱ	CHEMBL1681619
Drug and drug target database More...DrugBankⁱ	DB03727 Coproporphyrin I DB04461 Coproporphyrin Iii

Polymorphism and mutation 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

Acetylation

Proteomic databases

EPDⁱ	P06132
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P06132
PaxDbⁱ	P06132
PeptideAtlas More...PeptideAtlasⁱ	P06132
PRIDEⁱ	P06132
ProteomicsDBⁱ	51870
TopDownProteomicsⁱ	P06132

2D gel databases

USC-OGP 2-DE database More...OGPⁱ	P06132

OGPⁱ

P06132

PTM databases

iPTMnetⁱ	P06132
PhosphoSitePlusⁱ	P06132

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000126088 Expressed in 230 organ(s), highest expression level in liver
CleanExⁱ	HS_UROD
ExpressionAtlasⁱ	P06132 baseline and differential
Genevisibleⁱ	P06132 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA027468 HPA028668 HPA030350

HPAⁱ

HPA027468
HPA028668
HPA030350

Interactionⁱ

Subunit structureⁱ

Homodimer.3 Publications

Binary interactionsⁱ

With	Entry	#Exp.	IntAct	Notes
FAM84B	Q96KN1	5	EBI-2871776,EBI-9057780

With

Entry

#Exp.

IntAct

Notes

FAM84B

Q96KN1

5

EBI-2871776,EBI-9057780

Protein-protein interaction databases

BioGridⁱ	113235, 19 interactors
IntActⁱ	P06132, 12 interactors
Molecular INTeraction database More...MINTⁱ	P06132
STRINGⁱ	9606.ENSP00000246337

Structureⁱ

Secondary structure

Legend: HelixTurnBeta strandPDB Structure known for this area

Feature key	Position(s)	DescriptionActions	Length
Helixⁱ	18 – 24	Combined sources	7
Beta strandⁱ	38 – 40	Combined sources	3
Helixⁱ	44 – 51	Combined sources	8
Helixⁱ	55 – 59	Combined sources	5
Helixⁱ	62 – 75	Combined sources	14
Helixⁱ	89 – 93	Combined sources	5
Beta strandⁱ	99 – 101	Combined sources	3
Turnⁱ	102 – 104	Combined sources	3
Beta strandⁱ	105 – 107	Combined sources	3
Helixⁱ	115 – 120	Combined sources	6
Helixⁱ	124 – 126	Combined sources	3
Helixⁱ	127 – 130	Combined sources	4
Helixⁱ	132 – 145	Combined sources	14
Beta strandⁱ	151 – 156	Combined sources	6
Helixⁱ	158 – 167	Combined sources	10
Helixⁱ	175 – 183	Combined sources	9
Helixⁱ	185 – 208	Combined sources	24
Beta strandⁱ	212 – 218	Combined sources	7
Helixⁱ	221 – 223	Combined sources	3
Helixⁱ	226 – 232	Combined sources	7
Helixⁱ	234 – 250	Combined sources	17
Beta strandⁱ	258 – 262	Combined sources	5
Helixⁱ	266 – 268	Combined sources	3
Helixⁱ	269 – 272	Combined sources	4
Turnⁱ	273 – 276	Combined sources	4
Beta strandⁱ	278 – 281	Combined sources	4
Helixⁱ	288 – 295	Combined sources	8
Beta strandⁱ	297 – 305	Combined sources	9
Helixⁱ	307 – 311	Combined sources	5
Helixⁱ	314 – 328	Combined sources	15
Beta strandⁱ	330 – 339	Combined sources	10
Helixⁱ	347 – 365	Combined sources	19

Show more details

3D structure databases

Database of protein disorder More...DisProtⁱ	DP00308
ProteinModelPortalⁱ	P06132
SMRⁱ	P06132
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Miscellaneous databases

EvolutionaryTraceⁱ	P06132

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 COG0407 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000018302
HOVERGENⁱ	HBG000229
InParanoidⁱ	P06132
KEGG Orthology (KO) More...KOⁱ	K01599
OMAⁱ	SWAGQLS
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0EUA
PhylomeDBⁱ	P06132
TreeFamⁱ	TF300744

Family and domain databases

Conserved Domains Database More...CDDⁱ	cd00717 URO-D, 1 hit
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
Pfam protein domain database More...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

Sequence (1+)ⁱ

Sequence statusⁱ: Complete.

This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.Show all Align All

P06132-1 [UniParc]FASTA Add 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

Computationally mapped potential isoform sequencesⁱ

There are 4 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation

A0A1B0GVN9	A0A1B0GVN9_HUMAN	Uroporphyrinogen decarboxylase Uroporphyrinogen decarboxylase, EC 4.1.1.37	UROD	298	Annotation score:
A0A1B0GVZ4	A0A1B0GVZ4_HUMAN	Uroporphyrinogen decarboxylase Uroporphyrinogen decarboxylase	UROD	321	Annotation score:
Q5T446	Q5T446_HUMAN	Uroporphyrinogen decarboxylase Uroporphyrinogen decarboxylase	UROD	193	Annotation score:
H0Y5R6	H0Y5R6_HUMAN	Uroporphyrinogen decarboxylase Uroporphyrinogen decarboxylase	UROD	228	Annotation score:

Experimental Info

Feature key	Position(s)	DescriptionActions	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	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 Publication	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
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS518.1
PIRⁱ	A24411 G02786
NCBI Reference Sequences More...RefSeqⁱ	NP_000365.3, NM_000374.4
UniGeneⁱ	Hs.78601

Genome annotation databases

Ensemblⁱ	ENST00000246337; ENSP00000246337; ENSG00000126088
GeneIDⁱ	7389
KEGGⁱ	hsa:7389
UCSC genome browser More...UCSCⁱ	uc001cna.3 human

Similar proteinsⁱ

90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P06132	Uroporphyrinogen decarboxylase		MOUSE		367	UniRef90_P06132
Uroporphyrinogen decarboxylase		PONAB		367
Uroporphyrinogen decarboxylase		SHEEP		367
Uroporphyrinogen decarboxylase (Fragment)		RAT		364
Uroporphyrinogen decarboxylase		AILME		374
+148

Protein	Similar proteins		Species	Score	Length	Source
P06132	Uroporphyrinogen decarboxylase		MOUSE		367	UniRef50_P06132
Uroporphyrinogen decarboxylase		PONAB		367
Uroporphyrinogen decarboxylase		SHEEP		367
Uroporphyrinogen decarboxylase (Fragment)		RAT		364
Uroporphyrinogen decarboxylase		AILME		374
+290

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
UniGeneⁱ	Hs.78601

3D structure databases

Select the link destinations: Protein Data Bank Europe More...PDBeⁱ Protein Data Bank RCSB More...RCSB PDBⁱ Protein Data Bank Japan More...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	[»]
DisProtⁱ	DP00308
ProteinModelPortalⁱ	P06132
SMRⁱ	P06132
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	113235, 19 interactors
IntActⁱ	P06132, 12 interactors
MINTⁱ	P06132
STRINGⁱ	9606.ENSP00000246337

Chemistry databases

ChEMBLⁱ	CHEMBL1681619
DrugBankⁱ	DB03727 Coproporphyrin I DB04461 Coproporphyrin Iii

PTM databases

iPTMnetⁱ	P06132
PhosphoSitePlusⁱ	P06132

Polymorphism and mutation databases

BioMutaⁱ	UROD
DMDMⁱ	2507533

2D gel databases

OGPⁱ	P06132

OGPⁱ

P06132

Proteomic databases

EPDⁱ	P06132
MaxQBⁱ	P06132
PaxDbⁱ	P06132
PeptideAtlasⁱ	P06132
PRIDEⁱ	P06132
ProteomicsDBⁱ	51870
TopDownProteomicsⁱ	P06132

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	7389
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000246337; ENSP00000246337; ENSG00000126088
GeneIDⁱ	7389
KEGGⁱ	hsa:7389
UCSCⁱ	uc001cna.3 human

Organism-specific databases

CTDⁱ	7389
DisGeNETⁱ	7389
EuPathDBⁱ	HostDB:ENSG00000126088.12
GeneCardsⁱ	UROD
GeneReviewsⁱ	UROD
HGNCⁱ	HGNC:12591 UROD
HPAⁱ	HPA027468 HPA028668 HPA030350
MalaCardsⁱ	UROD
MIMⁱ	176100 phenotype 613521 gene
neXtProtⁱ	NX_P06132
OpenTargetsⁱ	ENSG00000126088
Orphanetⁱ	443062 Familial porphyria cutanea tarda 95159 Hepatoerythropoietic porphyria
PharmGKBⁱ	PA37221
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG2872 Eukaryota COG0407 LUCA
GeneTreeⁱ	ENSGT00390000018302
HOVERGENⁱ	HBG000229
InParanoidⁱ	P06132
KOⁱ	K01599
OMAⁱ	SWAGQLS
OrthoDBⁱ	EOG091G0EUA
PhylomeDBⁱ	P06132
TreeFamⁱ	TF300744

Enzyme and pathway databases

UniPathwayⁱ	UPA00251;UER00321
BioCycⁱ	MetaCyc:HS04993-MONOMER
BRENDAⁱ	4.1.1.37 2681
Reactomeⁱ	R-HSA-189451 Heme biosynthesis

Miscellaneous databases

ChiTaRSⁱ	UROD human
EvolutionaryTraceⁱ	P06132
GeneWikiⁱ	Uroporphyrinogen_III_decarboxylase
GenomeRNAiⁱ	7389
Protein Ontology More...PROⁱ	PR:P06132
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000126088 Expressed in 230 organ(s), highest expression level in liver
CleanExⁱ	HS_UROD
ExpressionAtlasⁱ	P06132 baseline and differential
Genevisibleⁱ	P06132 HS

Family and domain databases

CDDⁱ	cd00717 URO-D, 1 hit
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...

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:	November 7, 2018
	This is version 207 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, Complete proteome, Direct protein sequencing, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human chromosome 1
Human chromosome 1: entries, gene names and cross-references to MIM
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
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

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UniProtKB - P06132 (DCUP_HUMAN)

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Uroporphyrinogen decarboxylase

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<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: protoporphyrin-IX biosynthesis

Sites

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GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

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Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

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Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

2D gel databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

3D structure databases

Miscellaneous databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

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Experimental Info

Natural variant

Sequence databases

Genome annotation databases

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Sequence databases

3D structure databases

Protein-protein interaction databases

Chemistry databases

PTM databases

Polymorphism and mutation databases

2D gel databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

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Functionⁱ

Pathwayⁱ: protoporphyrin-IX biosynthesis

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Cross-referencesⁱ

Web resourcesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ