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UniProtKB - P06132 (DCUP_HUMAN)

Protein

Uroporphyrinogen decarboxylase

Gene

UROD

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

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<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

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

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<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

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 (hemE), Uroporphyrinogen decarboxylase (UROD), 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)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei55Substrate1
Binding sitei85Substrate1
Binding sitei86Substrate1
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections (‘Function’, ‘PTM / Processing’, ‘Pathology and Biotech’) according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei86Transition state stabilizer1
Binding sitei164Substrate1
Binding sitei219Substrate1
Binding sitei339Substrate1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

  • uroporphyrinogen decarboxylase activity Source: UniProtKB
View the complete GO annotation on QuickGO ...

GO - Biological processi

  • heme biosynthetic process Source: GO_Central
  • protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
View the complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionDecarboxylase, Lyase
Biological processHeme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...BioCyci		MetaCyc:HS04993-MONOMER

BRENDA Comprehensive Enzyme Information System

More...BRENDAi		4.1.1.37 2681

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-189451 Heme biosynthesis

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...UniPathwayi
UPA00251;UER00321

<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

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Uroporphyrinogen decarboxylase (EC:4.1.1.37)
Short name:
UPD
Short name:
URO-D
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:UROD
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 1

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...EuPathDBi		HostDB:ENSG00000126088.12

Human Gene Nomenclature Database

More...HGNCi		HGNC:12591 UROD

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		613521 gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P06132

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm

<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

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>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)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_02256725G → E in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs764268015Ensembl.1
Natural variantiVAR_02256980A → S in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs376921379Ensembl.1
Natural variantiVAR_010985142R → Q in FPCT. 1 PublicationCorresponds to variant dbSNP:rs1182234844Ensembl.1
Natural variantiVAR_022570144R → P in FPCT; decrease of activity. 1 Publication1
Natural variantiVAR_022571156G → D in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs762617943Ensembl.1
Natural variantiVAR_010986161L → Q in FPCT. 1 Publication1
Natural variantiVAR_007911165M → R in FPCT; activity < 2%. 2 PublicationsCorresponds to variant dbSNP:rs121918063EnsemblClinVar.1
Natural variantiVAR_022572193R → P in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs143823335EnsemblClinVar.1
Natural variantiVAR_007912195L → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918064EnsemblClinVar.1
Natural variantiVAR_022573216L → Q in FPCT. 1 Publication1
Natural variantiVAR_022574218E → K in FPCT; significant decrease of activity. 1 Publication1
Natural variantiVAR_010987219S → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs982293378Ensembl.1
Natural variantiVAR_009106229F → L in FPCT. 1 Publication1
Natural variantiVAR_022575232F → L in FPCT; decrease of activity. 1 Publication1
Natural variantiVAR_010988235P → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs141312224Ensembl.1
Natural variantiVAR_007913253L → Q in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs36033115EnsemblClinVar.1
Natural variantiVAR_022576260I → T in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs1483459837Ensembl.1
Natural variantiVAR_007716281G → V in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar.1
Natural variantiVAR_022577282L → R in FPCT. 1 Publication1
Natural variantiVAR_022578303G → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs964670864Ensembl.1
Natural variantiVAR_007914304N → K in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918065EnsemblClinVar.1
Natural variantiVAR_007915318G → R in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs116233118Ensembl.1
Natural variantiVAR_009108324M → T in FPCT. 1 Publication1
Natural variantiVAR_007916332R → H in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918066EnsemblClinVar.1
Natural variantiVAR_007917334I → T in FPCT. 1 Publication1
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)DescriptionActionsGraphical viewLength
Natural variantiVAR_02256846F → L in HEP; mild phenotype; strong decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs769378741Ensembl.1
Natural variantiVAR_00910362P → L in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918060EnsemblClinVar.1
Natural variantiVAR_00791080A → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs776907084Ensembl.1
Natural variantiVAR_065558168G → R in HEP; relative activity of 65% of wild-type towards uroporphyrinogen III. 1 Publication1
Natural variantiVAR_065559170G → D in HEP; relative activity of 17% and 60% of wild-type towards uroporphyrinogen I and III respectively. 1 Publication1
Natural variantiVAR_009105220H → P in HEP; mild form. 1 Publication1
Natural variantiVAR_007717292R → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918059EnsemblClinVar.1
Natural variantiVAR_009107311Y → C in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918061EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi86D → E: 5-10% of wild-type activity. 1 Publication1
Mutagenesisi86D → G: Very low activity. Binds substrate with similar geometry as wild-type. 1 Publication1
Mutagenesisi86D → N: No activity. Unable to bind substrate. 1 Publication1
Mutagenesisi164Y → F: 25-30% of wild-type activity. 1 Publication1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNET

More...DisGeNETi		7389

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...GeneReviewsi		UROD

MalaCards human disease database

More...MalaCardsi		UROD
MIMi176100 phenotype

Open Targets

More...OpenTargetsi		ENSG00000126088

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...Orphaneti		443062 Familial porphyria cutanea tarda
95159 Hepatoerythropoietic porphyria

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA37221

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...ChEMBLi		CHEMBL1681619

Drug and drug target database

More...DrugBanki		DB03727 Coproporphyrin I
DB04461 Coproporphyrin Iii

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		UROD

Domain mapping of disease mutations (DMDM)

More...DMDMi		2507533

<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

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001875691 – 367Uroporphyrinogen decarboxylaseAdd BLAST367

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei1N-acetylmethionineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P06132

MaxQB - The MaxQuant DataBase

More...MaxQBi		P06132

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		P06132

PeptideAtlas

More...PeptideAtlasi		P06132

PRoteomics IDEntifications database

More...PRIDEi		P06132

ProteomicsDB human proteome resource

More...ProteomicsDBi		51870

Consortium for Top Down Proteomics

More...TopDownProteomicsi		P06132

2D gel databases

USC-OGP 2-DE database

More...OGPi		P06132

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P06132

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		P06132

<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

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000126088 Expressed in 230 organ(s), highest expression level in liver

CleanEx database of gene expression profiles

More...CleanExi		HS_UROD

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P06132 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P06132 HS

Organism-specific databases

Human Protein Atlas

More...HPAi		HPA027468
HPA028668
HPA030350

<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

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homodimer.3 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

WithEntry#Exp.IntActNotes
FAM84BQ96KN15EBI-2871776,EBI-9057780

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...BioGridi		113235, 20 interactors

Protein interaction database and analysis system

More...IntActi		P06132, 12 interactors

Molecular INTeraction database

More...MINTi		P06132

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000246337

<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

1367
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Database of protein disorder

More...DisProti		DP00308

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

More...ProteinModelPortali		P06132

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		P06132

Database of comparative protein structure models

More...ModBasei		Search...

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...EvolutionaryTracei		P06132

<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

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni37 – 41Substrate binding5

<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

Belongs to the uroporphyrinogen decarboxylase family.Curated

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG2872 Eukaryota
COG0407 LUCA

Ensembl GeneTree

More...GeneTreei		ENSGT00390000018302

The HOVERGEN Database of Homologous Vertebrate Genes

More...HOVERGENi		HBG000229

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P06132

KEGG Orthology (KO)

More...KOi		K01599

Identification of Orthologs from Complete Genome Data

More...OMAi		SWAGQLS

Database of Orthologous Groups

More...OrthoDBi		EOG091G0EUA

Database for complete collections of gene phylogenies

More...PhylomeDBi		P06132

TreeFam database of animal gene trees

More...TreeFami		TF300744

Family and domain databases

Conserved Domains Database

More...CDDi		cd00717 URO-D, 1 hit

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		3.20.20.210, 1 hit

HAMAP database of protein families

More...HAMAPi		MF_00218 URO_D, 1 hit

Integrated resource of protein families, domains and functional sites

More...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

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF51726 SSF51726, 1 hit

TIGRFAMs; a protein family database

More...TIGRFAMsi		TIGR01464 hemE, 1 hit

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS00906 UROD_1, 1 hit
PS00907 UROD_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

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

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
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i840510B36CFC3856
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A1B0GVN9A0A1B0GVN9_HUMAN
Uroporphyrinogen decarboxylase
UROD
298Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A1B0GVZ4A0A1B0GVZ4_HUMAN
Uroporphyrinogen decarboxylase
UROD
321Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
Q5T446Q5T446_HUMAN
Uroporphyrinogen decarboxylase
UROD
193Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H0Y5R6H0Y5R6_HUMAN
Uroporphyrinogen decarboxylase
UROD
228Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti103G → S in AAA61258 (PubMed:3015909).Curated1
Sequence conflicti103G → S in AAB59456 (PubMed:2243121).Curated1
Sequence conflicti120R → A in AAA61258 (PubMed:3015909).Curated1
Sequence conflicti120R → A in AAB59456 (PubMed:2243121).Curated1
Sequence conflicti212 – 214Missing in AAB59456 (PubMed:2243121).Curated3

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06068315K → E. Corresponds to variant dbSNP:rs11541959Ensembl.1
Natural variantiVAR_02256725G → E in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs764268015Ensembl.1
Natural variantiVAR_02256846F → L in HEP; mild phenotype; strong decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs769378741Ensembl.1
Natural variantiVAR_00910362P → L in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918060EnsemblClinVar.1
Natural variantiVAR_06745777P → L3 PublicationsCorresponds to variant dbSNP:rs1131147Ensembl.1
Natural variantiVAR_00791080A → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs776907084Ensembl.1
Natural variantiVAR_02256980A → S in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs376921379Ensembl.1
Natural variantiVAR_060684106P → L. Corresponds to variant dbSNP:rs11541962Ensembl.1
Natural variantiVAR_060685113R → T. Corresponds to variant dbSNP:rs11541963Ensembl.1
Natural variantiVAR_009104134V → Q in FPCT and HEP; requires 2 nucleotide substitutions; nearly normal activity. 4 Publications1
Natural variantiVAR_010985142R → Q in FPCT. 1 PublicationCorresponds to variant dbSNP:rs1182234844Ensembl.1
Natural variantiVAR_022570144R → P in FPCT; decrease of activity. 1 Publication1
Natural variantiVAR_022571156G → D in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs762617943Ensembl.1
Natural variantiVAR_010986161L → Q in FPCT. 1 Publication1
Natural variantiVAR_007911165M → R in FPCT; activity < 2%. 2 PublicationsCorresponds to variant dbSNP:rs121918063EnsemblClinVar.1
Natural variantiVAR_007714167E → K in HEP and FPCT; nearly normal activity. 2 PublicationsCorresponds to variant dbSNP:rs121918058EnsemblClinVar.1
Natural variantiVAR_065558168G → R in HEP; relative activity of 65% of wild-type towards uroporphyrinogen III. 1 Publication1
Natural variantiVAR_065559170G → D in HEP; relative activity of 17% and 60% of wild-type towards uroporphyrinogen I and III respectively. 1 Publication1
Natural variantiVAR_022572193R → P in FPCT; insoluble protein. 1 PublicationCorresponds to variant dbSNP:rs143823335EnsemblClinVar.1
Natural variantiVAR_007912195L → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918064EnsemblClinVar.1
Natural variantiVAR_022573216L → Q in FPCT. 1 Publication1
Natural variantiVAR_022574218E → K in FPCT; significant decrease of activity. 1 Publication1
Natural variantiVAR_010987219S → F in FPCT. 1 PublicationCorresponds to variant dbSNP:rs982293378Ensembl.1
Natural variantiVAR_009105220H → P in HEP; mild form. 1 Publication1
Natural variantiVAR_009106229F → L in FPCT. 1 Publication1
Natural variantiVAR_022575232F → L in FPCT; decrease of activity. 1 Publication1
Natural variantiVAR_010988235P → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs141312224Ensembl.1
Natural variantiVAR_007913253L → Q in FPCT; decrease of activity. 2 PublicationsCorresponds to variant dbSNP:rs36033115EnsemblClinVar.1
Natural variantiVAR_022576260I → T in FPCT; decrease of activity. 1 PublicationCorresponds to variant dbSNP:rs1483459837Ensembl.1
Natural variantiVAR_007715281G → E in FPCT and HEP. 3 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar.1
Natural variantiVAR_007716281G → V in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs121918057EnsemblClinVar.1
Natural variantiVAR_022577282L → R in FPCT. 1 Publication1
Natural variantiVAR_007717292R → G in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918059EnsemblClinVar.1
Natural variantiVAR_022578303G → S in FPCT. 1 PublicationCorresponds to variant dbSNP:rs964670864Ensembl.1
Natural variantiVAR_060686303G → V2 PublicationsCorresponds to variant dbSNP:rs17849533Ensembl.1
Natural variantiVAR_007914304N → K in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918065EnsemblClinVar.1
Natural variantiVAR_009107311Y → C in HEP. 1 PublicationCorresponds to variant dbSNP:rs121918061EnsemblClinVar.1
Natural variantiVAR_007915318G → R in FPCT. 2 PublicationsCorresponds to variant dbSNP:rs116233118Ensembl.1
Natural variantiVAR_009108324M → T in FPCT. 1 Publication1
Natural variantiVAR_007916332R → H in FPCT. 1 PublicationCorresponds to variant dbSNP:rs121918066EnsemblClinVar.1
Natural variantiVAR_007917334I → T in FPCT. 1 Publication1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...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

Protein sequence database of the Protein Information Resource

More...PIRi		A24411
G02786

NCBI Reference Sequences

More...RefSeqi		NP_000365.3, NM_000374.4

UniGene gene-oriented nucleotide sequence clusters

More...UniGenei		Hs.78601

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000246337; ENSP00000246337; ENSG00000126088

Database of genes from NCBI RefSeq genomes

More...GeneIDi		7389

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:7389

UCSC genome browser

More...UCSCi		uc001cna.3 human

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>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
CCDSiCCDS518.1
PIRiA24411
G02786
RefSeqiNP_000365.3, NM_000374.4
UniGeneiHs.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 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
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113235, 20 interactors
IntActiP06132, 12 interactors
MINTiP06132
STRINGi9606.ENSP00000246337

Chemistry databases

ChEMBLiCHEMBL1681619
DrugBankiDB03727 Coproporphyrin I
DB04461 Coproporphyrin Iii

PTM databases

iPTMnetiP06132
PhosphoSitePlusiP06132

Polymorphism and mutation databases

BioMutaiUROD
DMDMi2507533

2D gel databases

OGPiP06132

Proteomic databases

EPDiP06132
MaxQBiP06132
PaxDbiP06132
PeptideAtlasiP06132
PRIDEiP06132
ProteomicsDBi51870
TopDownProteomicsiP06132

Protocols and materials databases

The DNASU plasmid repository

More...DNASUi		7389
Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		7389
DisGeNETi7389
EuPathDBiHostDB:ENSG00000126088.12

GeneCards: human genes, protein and diseases

More...GeneCardsi		UROD
GeneReviewsiUROD
HGNCiHGNC:12591 UROD
HPAiHPA027468
HPA028668
HPA030350
MalaCardsiUROD
MIMi176100 phenotype
613521 gene
neXtProtiNX_P06132
OpenTargetsiENSG00000126088
Orphaneti443062 Familial porphyria cutanea tarda
95159 Hepatoerythropoietic porphyria
PharmGKBiPA37221

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG2872 Eukaryota
COG0407 LUCA
GeneTreeiENSGT00390000018302
HOVERGENiHBG000229
InParanoidiP06132
KOiK01599
OMAiSWAGQLS
OrthoDBiEOG091G0EUA
PhylomeDBiP06132
TreeFamiTF300744

Enzyme and pathway databases

UniPathwayi
UPA00251;UER00321

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

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...ChiTaRSi		UROD human
EvolutionaryTraceiP06132

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		Uroporphyrinogen_III_decarboxylase

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		7389

Protein Ontology

More...PROi		PR:P06132

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000126088 Expressed in 230 organ(s), highest expression level in liver
CleanExiHS_UROD
ExpressionAtlasiP06132 baseline and differential
GenevisibleiP06132 HS

Family and domain databases

CDDicd00717 URO-D, 1 hit
Gene3Di3.20.20.210, 1 hit
HAMAPiMF_00218 URO_D, 1 hit
InterProiView protein in InterPro
IPR038071 UROD/MetE-like_sf
IPR006361 Uroporphyrinogen_deCO2ase_HemE
IPR000257 Uroporphyrinogen_deCOase
PfamiView protein in Pfam
PF01208 URO-D, 1 hit
SUPFAMiSSF51726 SSF51726, 1 hit
TIGRFAMsiTIGR01464 hemE, 1 hit
PROSITEiView protein in PROSITE
PS00906 UROD_1, 1 hit
PS00907 UROD_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...ProtoNeti		Search...

<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 subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiDCUP_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P06132
Secondary accession number(s): A8K762
, Q16863, Q16883, Q53YB8, Q53ZP6, Q6IB28, Q9BUZ0
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: November 1, 1997
Last modified: December 5, 2018
This is version 208 of the entry and version 2 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<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

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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  3. SIMILARITY comments
    Index of protein domains and families
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  7. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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