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UniProtKB - P36551 (HEM6_HUMAN)

Entry version 207 (02 Jun 2021)
Sequence version 3 (07 Jun 2005)
Previous versions | rss
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Protein

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

Gene

CPOX

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

Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">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%5Fsection">'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 1 of the subpathway that synthesizes protoporphyrinogen-IX from coproporphyrinogen-III (O2 route). 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 protoporphyrinogen-IX from coproporphyrinogen-III (O2 route), the pathway protoporphyrin-IX biosynthesis and in Porphyrin-containing compound metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> 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 sitei244SubstrateCurated1
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei258Proton donor1 Publication1
<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>Sitei327Important for dimerization1

<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

Complete GO annotation on QuickGO ...

GO - Biological processi

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 functionOxidoreductase
Biological processHeme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...BioCyci		MetaCyc:HS01369-MONOMER

BRENDA Comprehensive Enzyme Information System

More...BRENDAi		1.3.3.3, 2681

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		P36551

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;UER00322

<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%5Fand%5Ftaxonomy%5Fsection">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:
Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial (EC:1.3.3.3)
Short name:
COX
Short name:
Coprogen oxidase
Short name:
Coproporphyrinogenase
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">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:CPOX
Synonyms:CPO, CPX
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">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%5Fand%5Ftaxonomy%5Fsection">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%5Fand%5Ftaxonomy%5Fsection">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%5Fand%5Ftaxonomy%5Fsection">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%5Fmanual">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 3

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:2321, CPOX

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		612732, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P36551

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000080819.6

<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

Keywords - Cellular componenti

Mitochondrion

<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

Hereditary coproporphyria (HCP)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. Hereditary coproporphyria is an acute hepatic porphyria characterized by skin photosensitivity, attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Hereditary coproporphyria is biochemically characterized by overexcretion of coproporphyrin III in the urine and in the feces.
Related information in OMIM
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_08451129 – 454Missing in HCP. 1 PublicationAdd BLAST426
Natural variantiVAR_023444135V → A in HCP. 1 PublicationCorresponds to variant dbSNP:rs201826432Ensembl.1
Natural variantiVAR_002151162 – 168Missing in HCP. 1 Publication7
Natural variantiVAR_002152189G → S in HCP; <5% of activity. 1 PublicationCorresponds to variant dbSNP:rs759347283Ensembl.1
Natural variantiVAR_002153197G → W in HCP. 1 Publication1
Natural variantiVAR_002154201E → K in HCP. 1 PublicationCorresponds to variant dbSNP:rs1374394802Ensembl.1
Natural variantiVAR_019067208S → F in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917872EnsemblClinVar.1
Natural variantiVAR_023445214L → R in HCP. 1 Publication1
Natural variantiVAR_023446249P → R in HCP. 1 Publication1
Natural variantiVAR_002155249P → S in HCP. 1 Publication1
Natural variantiVAR_058005279G → R in HCP; a patient carrying also the L-12 mutation in ALAD. 1 PublicationCorresponds to variant dbSNP:rs121917874EnsemblClinVar.1
Natural variantiVAR_002157280G → R in HCP. 1 Publication1
Natural variantiVAR_002159295H → D in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917870EnsemblClinVar.1
Natural variantiVAR_019068328R → C in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917873EnsemblClinVar.1
Natural variantiVAR_002160331R → W in HCP. 2 PublicationsCorresponds to variant dbSNP:rs121917866EnsemblClinVar.1
Natural variantiVAR_002161390Missing in HCP. 1 Publication1
Natural variantiVAR_002163427W → R in HCP. 1 Publication1
Natural variantiVAR_019069447R → C in HCP. 1 PublicationCorresponds to variant dbSNP:rs28931603EnsemblClinVar.1
Harderoporphyria (HARPO)2 Publications
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. HARPO is a rare erythropoietic variant form characterized by neonatal hemolytic anemia, sometimes accompanied by skin lesions, and massive excretion of harderoporphyrin in feces.
Related information in OMIM
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_084512327H → R in HARPO. 1 Publication1
Natural variantiVAR_002162404K → E in HARPO. 1 PublicationCorresponds to variant dbSNP:rs121917868EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'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>Mutagenesisi392 – 418Missing : Loss for dimerization. 1 PublicationAdd BLAST27

Keywords - Diseasei

Disease variant, Hereditary hemolytic anemia

Organism-specific databases

DisGeNET

More...DisGeNETi		1371

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

More...GeneReviewsi		CPOX

MalaCards human disease database

More...MalaCardsi		CPOX
MIMi121300, phenotype
618892, phenotype

Open Targets

More...OpenTargetsi		ENSG00000080819

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

More...Orphaneti		79273, Hereditary coproporphyria

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA134979958

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		P36551, Tbio

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...ChEMBLi		CHEMBL1681618

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		CPOX

Domain mapping of disease mutations (DMDM)

More...DMDMi		67476671

<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 transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 110MitochondrionBy similarityAdd BLAST110
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_0000006029111 – 454Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrialAdd BLAST344

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 residuei112PhosphoserineBy similarity1
Modified residuei404N6-acetyllysine; alternateBy similarity1
Modified residuei404N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P36551

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P36551

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		P36551

MaxQB - The MaxQuant DataBase

More...MaxQBi		P36551

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

More...PaxDbi		P36551

PeptideAtlas

More...PeptideAtlasi		P36551

PRoteomics IDEntifications database

More...PRIDEi		P36551

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		55213 [P36551-1]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P36551

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

More...PhosphoSitePlusi		P36551

SwissPalm database of S-palmitoylation events

More...SwissPalmi		P36551

<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		ENSG00000080819, Expressed in liver and 224 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P36551, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P36551, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000080819, Tissue enhanced (brain)

<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%5Fsection">'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%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homodimer.

1 Publication

GO - Molecular functioni

Complete GO annotation on QuickGO ...

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		107763, 51 interactors

Protein interaction database and analysis system

More...IntActi		P36551, 24 interactors

Molecular INTeraction database

More...MINTi		P36551

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000264193

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		P36551, protein

<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

1454
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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

More...SMRi		P36551

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...EvolutionaryTracei		P36551

<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>Regioni43 – 70DisorderedSequence analysisAdd BLAST28
Regioni193 – 202Important for dimerizationCurated10
Regioni260 – 262Substrate binding3
Regioni392 – 428Important for dimerizationAdd BLAST37
Regioni411 – 413Substrate binding3

<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 aerobic coproporphyrinogen-III oxidase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG1518, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00390000017311

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		CLU_026169_1_0_1

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P36551

Identification of Orthologs from Complete Genome Data

More...OMAi		HVQRAEM

Database of Orthologous Groups

More...OrthoDBi		1080991at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P36551

TreeFam database of animal gene trees

More...TreeFami		TF300703

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		3.40.1500.10, 1 hit

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR001260, Coprogen_oxidase_aer
IPR036406, Coprogen_oxidase_aer_sf
IPR018375, Coprogen_oxidase_CS

The PANTHER Classification System

More...PANTHERi		PTHR10755, PTHR10755, 1 hit

Pfam protein domain database

More...Pfami		View protein in Pfam
PF01218, Coprogen_oxidas, 1 hit

Protein Motif fingerprint database; a protein domain database

More...PRINTSi		PR00073, COPRGNOXDASE

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF102886, SSF102886, 1 hit

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS01021, COPROGEN_OXIDASE, 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%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">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.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform 1 (identifier: P36551-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MALQLGRLSS GPCWLVARGG CGGPRAWSQC GGGGLRAWSQ RSAAGRVCRP 
        60         70         80         90        100
PGPAGTEQSR GLGHGSTSRG GPWVGTGLAA ALAGLVGLAT AAFGHVQRAE 
       110        120        130        140        150
MLPKTSGTRA TSLGRPEEEE DELAHRCSSF MAPPVTDLGE LRRRPGDMKT 
       160        170        180        190        200
KMELLILETQ AQVCQALAQV DGGANFSVDR WERKEGGGGI SCVLQDGCVF 
       210        220        230        240        250
EKAGVSISVV HGNLSEEAAK QMRSRGKVLK TKDGKLPFCA MGVSSVIHPK 
       260        270        280        290        300
NPHAPTIHFN YRYFEVEEAD GNKQWWFGGG CDLTPTYLNQ EDAVHFHRTL 
       310        320        330        340        350
KEACDQHGPD LYPKFKKWCD DYFFIAHRGE RRGIGGIFFD DLDSPSKEEV 
       360        370        380        390        400
FRFVQSCARA VVPSYIPLVK KHCDDSFTPQ EKLWQQLRRG RYVEFNLLYD 
       410        420        430        440        450
RGTKFGLFTP GSRIESILMS LPLTARWEYM HSPSENSKEA EILEVLRHPR 

DWVR
Length:454
Mass (Da):50,152
Last modified:June 7, 2005 - v3
<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:i6EC3D15FD8FD86B5
GO
Isoform 2 (identifier: P36551-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     272-287: NKQWWFGGGCDLTPTY → KGLRSYGKYCRAKCAF
     288-454: Missing.

Show »
Length:287
Mass (Da):30,383
Checksum:i69DFD4AC8247AE4E
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 is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D6RER6D6RER6_HUMAN
Oxygen-dependent coproporphyrinogen...
CPOX
175Annotation 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>

<p>This subsection of the 'Sequence' section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence BAA04033 differs from that shown. Reason: Erroneous initiation. Truncated N-terminus.Curated

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 conflicti247I → T in CAA82250 (PubMed:8159699).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_08451129 – 454Missing in HCP. 1 PublicationAdd BLAST426
Natural variantiVAR_023444135V → A in HCP. 1 PublicationCorresponds to variant dbSNP:rs201826432Ensembl.1
Natural variantiVAR_002151162 – 168Missing in HCP. 1 Publication7
Natural variantiVAR_002152189G → S in HCP; <5% of activity. 1 PublicationCorresponds to variant dbSNP:rs759347283Ensembl.1
Natural variantiVAR_002153197G → W in HCP. 1 Publication1
Natural variantiVAR_002154201E → K in HCP. 1 PublicationCorresponds to variant dbSNP:rs1374394802Ensembl.1
Natural variantiVAR_019067208S → F in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917872EnsemblClinVar.1
Natural variantiVAR_023445214L → R in HCP. 1 Publication1
Natural variantiVAR_023446249P → R in HCP. 1 Publication1
Natural variantiVAR_002155249P → S in HCP. 1 Publication1
Natural variantiVAR_002156272N → H2 PublicationsCorresponds to variant dbSNP:rs1131857EnsemblClinVar.1
Natural variantiVAR_058005279G → R in HCP; a patient carrying also the L-12 mutation in ALAD. 1 PublicationCorresponds to variant dbSNP:rs121917874EnsemblClinVar.1
Natural variantiVAR_002157280G → R in HCP. 1 Publication1
Natural variantiVAR_002158294V → I3 PublicationsCorresponds to variant dbSNP:rs2228056EnsemblClinVar.1
Natural variantiVAR_002159295H → D in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917870EnsemblClinVar.1
Natural variantiVAR_084512327H → R in HARPO. 1 Publication1
Natural variantiVAR_019068328R → C in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917873EnsemblClinVar.1
Natural variantiVAR_002160331R → W in HCP. 2 PublicationsCorresponds to variant dbSNP:rs121917866EnsemblClinVar.1
Natural variantiVAR_048827352R → C. Corresponds to variant dbSNP:rs11921054EnsemblClinVar.1
Natural variantiVAR_002161390Missing in HCP. 1 Publication1
Natural variantiVAR_002162404K → E in HARPO. 1 PublicationCorresponds to variant dbSNP:rs121917868EnsemblClinVar.1
Natural variantiVAR_002163427W → R in HCP. 1 Publication1
Natural variantiVAR_019069447R → C in HCP. 1 PublicationCorresponds to variant dbSNP:rs28931603EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_057182272 – 287NKQWW…LTPTY → KGLRSYGKYCRAKCAF in isoform 2. 1 PublicationAdd BLAST16
Alternative sequenceiVSP_057183288 – 454Missing in isoform 2. 1 PublicationAdd BLAST167

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
Z34531 Z34808 Genomic DNA Translation: CAA84292.1
AK290140 mRNA Translation: BAF82829.1
AK299692 mRNA Translation: BAG61597.1
AK223481 mRNA Translation: BAD97201.1
CH471052 Genomic DNA Translation: EAW79854.1
BC017210 mRNA Translation: AAH17210.1
BC023551 mRNA Translation: AAH23551.1
BC023554 mRNA Translation: AAH23554.1
D16611 mRNA Translation: BAA04033.1 Different initiation.
Z28409 mRNA Translation: CAA82250.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS2932.1 [P36551-1]

Protein sequence database of the Protein Information Resource

More...PIRi		I52444

NCBI Reference Sequences

More...RefSeqi		NP_000088.3, NM_000097.5 [P36551-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000647941; ENSP00000497326; ENSG00000080819 [P36551-1]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		1371

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:1371

UCSC genome browser

More...UCSCi		uc003dsx.4, human [P36551-1]

Keywords - Coding sequence diversityi

Alternative splicing

<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

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z34531 Z34808 Genomic DNA Translation: CAA84292.1
AK290140 mRNA Translation: BAF82829.1
AK299692 mRNA Translation: BAG61597.1
AK223481 mRNA Translation: BAD97201.1
CH471052 Genomic DNA Translation: EAW79854.1
BC017210 mRNA Translation: AAH17210.1
BC023551 mRNA Translation: AAH23551.1
BC023554 mRNA Translation: AAH23554.1
D16611 mRNA Translation: BAA04033.1 Different initiation.
Z28409 mRNA Translation: CAA82250.1
CCDSiCCDS2932.1 [P36551-1]
PIRiI52444
RefSeqiNP_000088.3, NM_000097.5 [P36551-1]

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
2AEXX-ray1.58A111-454[»]
SMRiP36551
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi107763, 51 interactors
IntActiP36551, 24 interactors
MINTiP36551
STRINGi9606.ENSP00000264193

Chemistry databases

ChEMBLiCHEMBL1681618

PTM databases

iPTMnetiP36551
PhosphoSitePlusiP36551
SwissPalmiP36551

Genetic variation databases

BioMutaiCPOX
DMDMi67476671

Proteomic databases

EPDiP36551
jPOSTiP36551
MassIVEiP36551
MaxQBiP36551
PaxDbiP36551
PeptideAtlasiP36551
PRIDEiP36551
ProteomicsDBi55213 [P36551-1]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		2788, 164 antibodies

The DNASU plasmid repository

More...DNASUi		1371

Genome annotation databases

EnsembliENST00000647941; ENSP00000497326; ENSG00000080819 [P36551-1]
GeneIDi1371
KEGGihsa:1371
UCSCiuc003dsx.4, human [P36551-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		1371
DisGeNETi1371

GeneCards: human genes, protein and diseases

More...GeneCardsi		CPOX
GeneReviewsiCPOX
HGNCiHGNC:2321, CPOX
HPAiENSG00000080819, Tissue enhanced (brain)
MalaCardsiCPOX
MIMi121300, phenotype
612732, gene
618892, phenotype
neXtProtiNX_P36551
OpenTargetsiENSG00000080819
Orphaneti79273, Hereditary coproporphyria
PharmGKBiPA134979958
VEuPathDBiHostDB:ENSG00000080819.6

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG1518, Eukaryota
GeneTreeiENSGT00390000017311
HOGENOMiCLU_026169_1_0_1
InParanoidiP36551
OMAiHVQRAEM
OrthoDBi1080991at2759
PhylomeDBiP36551
TreeFamiTF300703

Enzyme and pathway databases

UniPathwayiUPA00251;UER00322
BioCyciMetaCyc:HS01369-MONOMER
BRENDAi1.3.3.3, 2681
PathwayCommonsiP36551
ReactomeiR-HSA-189451, Heme biosynthesis

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		1371, 199 hits in 1007 CRISPR screens

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

More...ChiTaRSi		CPOX, human
EvolutionaryTraceiP36551

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		Coproporphyrinogen_III_oxidase

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

More...GenomeRNAii		1371
PharosiP36551, Tbio

Protein Ontology

More...PROi		PR:P36551
RNActiP36551, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000080819, Expressed in liver and 224 other tissues
ExpressionAtlasiP36551, baseline and differential
GenevisibleiP36551, HS

Family and domain databases

Gene3Di3.40.1500.10, 1 hit
InterProiView protein in InterPro
IPR001260, Coprogen_oxidase_aer
IPR036406, Coprogen_oxidase_aer_sf
IPR018375, Coprogen_oxidase_CS
PANTHERiPTHR10755, PTHR10755, 1 hit
PfamiView protein in Pfam
PF01218, Coprogen_oxidas, 1 hit
PRINTSiPR00073, COPRGNOXDASE
SUPFAMiSSF102886, SSF102886, 1 hit
PROSITEiView protein in PROSITE
PS01021, COPROGEN_OXIDASE, 1 hit

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		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 nameiHEM6_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: P36551
Secondary accession number(s): A8K275
, B4DSD5, Q14060, Q53F08, Q8IZ45, Q96AF3
<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%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: June 7, 2005
Last modified: June 2, 2021
This is version 207 of the entry and version 3 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, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  2. Human entries with genetic variants
    List of human entries with genetic variants
  3. Human variants curated from literature reports
    Index of human variants curated from literature reports
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families
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