CPOX

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.

Catalytic activityⁱ

Coproporphyrinogen-III + O₂ + 2 H⁺ = protoporphyrinogen-IX + 2 CO₂ + 2 H₂O.

Pathwayⁱ: protoporphyrin-IX biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes protoporphyrinogen-IX from coproporphyrinogen-III (O2 route).
Proteins known to be involved in this subpathway in this organism are:

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial (CPOX)

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 key	Position(s)	DescriptionActions	Length
Binding siteⁱ	244	SubstrateCurated	1
Active siteⁱ	258	Proton donor1 Publication	1
Siteⁱ	327	Important for dimerization	1

GO - Molecular functionⁱ

coproporphyrinogen oxidase activity
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
protein homodimerization activity
Source: UniProtKB
Inferred from direct assayⁱ
- 16176984

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

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

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Oxidoreductase
Biological process	Heme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

BioCycⁱ	MetaCyc:HS01369-MONOMER
BRENDAⁱ	1.3.3.3 2681
Reactomeⁱ	R-HSA-189451 Heme biosynthesis
UniPathwayⁱ	UPA00251;UER00322

Names & Taxonomyⁱ

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
Gene namesⁱ	Name:CPOX Synonyms:CPO, CPX
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 3

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000080819.6
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:2321 CPOX
MIMⁱ	612732 gene
neXtProtⁱ	NX_P36551

Keywords - Cellular componentⁱ

Mitochondrion

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Hereditary coproporphyria (HCP)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. 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.

Mutagenesis

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Mutagenesisⁱ	392 – 418	Missing : Loss for dimerization. 1 PublicationAdd BLAST		27

Keywords - Diseaseⁱ

Disease mutation

Organism-specific databases

DisGeNET More...DisGeNETⁱ	1371
GeneReviewsⁱ	CPOX
MalaCards human disease database More...MalaCardsⁱ	CPOX
MIMⁱ	121300 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000080819
Orphanetⁱ	79273 Hereditary coproporphyria
PharmGKBⁱ	PA134979958

Chemistry databases

ChEMBLⁱ	CHEMBL1681618

ChEMBLⁱ

CHEMBL1681618

Polymorphism and mutation databases

BioMutaⁱ	CPOX
DMDMⁱ	67476671

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transit peptideⁱ	1 – 110	MitochondrionBy similarityAdd BLAST		110
ChainⁱPRO_0000006029	111 – 454	Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrialAdd BLAST		344

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Modified residueⁱ	112	PhosphoserineBy similarity	1
Modified residueⁱ	404	N6-acetyllysine; alternateBy similarity	1
Modified residueⁱ	404	N6-succinyllysine; alternateBy similarity	1

Keywords - PTMⁱ

Acetylation, Phosphoprotein

Proteomic databases

EPDⁱ	P36551
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P36551
PaxDbⁱ	P36551
PeptideAtlas More...PeptideAtlasⁱ	P36551
PRIDEⁱ	P36551
ProteomicsDBⁱ	55213

PTM databases

iPTMnetⁱ	P36551
PhosphoSitePlusⁱ	P36551
SwissPalmⁱ	P36551

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000080819 Expressed in 214 organ(s), highest expression level in liver
CleanExⁱ	HS_CPO HS_CPOX
ExpressionAtlasⁱ	P36551 baseline and differential
Genevisibleⁱ	P36551 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA015736 HPA054448

HPAⁱ

HPA015736
HPA054448

Interactionⁱ

Subunit structureⁱ

Homodimer.1 Publication

GO - Molecular functionⁱ

protein homodimerization activity
Source: UniProtKB
Inferred from direct assayⁱ
- 16176984

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	107763, 14 interactors
IntActⁱ	P36551, 21 interactors
Molecular INTeraction database More...MINTⁱ	P36551
STRINGⁱ	9606.ENSP00000264193

Structureⁱ

Secondary structure

Legend: HelixTurnBeta strandPDB Structure known for this area

Feature key	Position(s)	DescriptionActions	Length
Helixⁱ	122 – 128	Combined sources	7
Beta strandⁱ	130 – 132	Combined sources	3
Helixⁱ	138 – 143	Combined sources	6
Helixⁱ	148 – 170	Combined sources	23
Beta strandⁱ	177 – 183	Combined sources	7
Turnⁱ	184 – 186	Combined sources	3
Beta strandⁱ	187 – 196	Combined sources	10
Beta strandⁱ	198 – 213	Combined sources	16
Helixⁱ	216 – 224	Combined sources	9
Beta strandⁱ	237 – 251	Combined sources	15
Beta strandⁱ	256 – 267	Combined sources	12
Beta strandⁱ	273 – 284	Combined sources	12
Helixⁱ	290 – 305	Combined sources	16
Helixⁱ	311 – 322	Combined sources	12
Helixⁱ	326 – 328	Combined sources	3
Beta strandⁱ	330 – 342	Combined sources	13
Helixⁱ	347 – 359	Combined sources	13
Helixⁱ	361 – 372	Combined sources	12
Helixⁱ	379 – 399	Combined sources	21
Helixⁱ	406 – 408	Combined sources	3
Beta strandⁱ	409 – 411	Combined sources	3
Helixⁱ	414 – 420	Combined sources	7
Helixⁱ	438 – 447	Combined sources	10

Show more details

3D structure databases

ProteinModelPortalⁱ	P36551
SMRⁱ	P36551
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Miscellaneous databases

EvolutionaryTraceⁱ	P36551

EvolutionaryTraceⁱ

P36551

Family & Domainsⁱ

Region

Feature key	Position(s)	DescriptionActions	Length
Regionⁱ	193 – 202	Important for dimerizationCurated	10
Regionⁱ	260 – 262	Substrate binding	3
Regionⁱ	392 – 428	Important for dimerizationAdd BLAST	37
Regionⁱ	411 – 413	Substrate binding	3

Sequence similaritiesⁱ

Belongs to the aerobic coproporphyrinogen-III oxidase family.Curated

Keywords - Domainⁱ

Transit peptide

Phylogenomic databases

eggNOGⁱ	KOG1518 Eukaryota COG0408 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000017311
HOGENOMⁱ	HOG000262768
HOVERGENⁱ	HBG051897
InParanoidⁱ	P36551
KEGG Orthology (KO) More...KOⁱ	K00228
OMAⁱ	MDLTPYY
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G06ZF
PhylomeDBⁱ	P36551
TreeFamⁱ	TF300703

Family and domain databases

Gene3Dⁱ	3.40.1500.10, 1 hit
InterProⁱ	View protein in InterPro IPR001260 Coprogen_oxidase_aer IPR036406 Coprogen_oxidase_aer_sf IPR018375 Coprogen_oxidase_CS
PANTHERⁱ	PTHR10755 PTHR10755, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF01218 Coprogen_oxidas, 1 hit
PRINTSⁱ	PR00073 COPRGNOXDASE
SUPFAMⁱ	SSF102886 SSF102886, 1 hit
PROSITEⁱ	View protein in PROSITE PS01021 COPROGEN_OXIDASE, 1 hit

Sequences (2+)ⁱ

Sequence statusⁱ: Complete.

Sequence processingⁱ: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsⁱ produced by alternative splicing. Align Add to basket

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show all Align All

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

This isoform has been chosen as the 'canonical' 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

Checksum:ⁱ6EC3D15FD8FD86B5

GO

Isoform 2 (identifier: P36551-2) [UniParc]FASTA Add to basket

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

« 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 
NPHAPTIHFN YRYFEVEEAD GKGLRSYGKY CRAKCAF

Note: No experimental confirmation available.

Show »

Length:287

Mass (Da):30,383

Checksum:ⁱ69DFD4AC8247AE4E

GO

Computationally mapped potential isoform sequencesⁱ

There are 2 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation

D6RER6	D6RER6_HUMAN	Oxygen-dependent coproporphyrinogen... Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial	CPOX	175	Annotation score:
H0YA22	H0YA22_HUMAN	Oxygen-dependent coproporphyrinogen... Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial	CPOX	70	Annotation score:

Sequence cautionⁱ

The sequence BAA04033 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	247	I → T in CAA82250 (PubMed:8159699).Curated		1

Natural variant

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_023444	135	V → A in HCP. 1 PublicationCorresponds to variant dbSNP:rs201826432Ensembl.	1
Natural variantⁱVAR_002151	162 – 168	Missing in HCP. 1 Publication	7
Natural variantⁱVAR_002152	189	G → S in HCP; <5% of activity. 1 PublicationCorresponds to variant dbSNP:rs759347283Ensembl.	1
Natural variantⁱVAR_002153	197	G → W in HCP. 1 Publication	1
Natural variantⁱVAR_002154	201	E → K in HCP. 1 Publication	1
Natural variantⁱVAR_019067	208	S → F in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917872EnsemblClinVar.	1
Natural variantⁱVAR_023445	214	L → R in HCP. 1 Publication	1
Natural variantⁱVAR_023446	249	P → R in HCP. 1 Publication	1
Natural variantⁱVAR_002155	249	P → S in HCP. 1 Publication	1
Natural variantⁱVAR_002156	272	N → H2 PublicationsCorresponds to variant dbSNP:rs1131857EnsemblClinVar.	1
Natural variantⁱVAR_058005	279	G → R in HCP; a patient carrying also the L-12 mutation in ALAD. 1 PublicationCorresponds to variant dbSNP:rs121917874EnsemblClinVar.	1
Natural variantⁱVAR_002157	280	G → R in HCP. 1 Publication	1
Natural variantⁱVAR_002158	294	V → I3 PublicationsCorresponds to variant dbSNP:rs2228056EnsemblClinVar.	1
Natural variantⁱVAR_002159	295	H → D in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917870EnsemblClinVar.	1
Natural variantⁱVAR_019068	328	R → C in HCP. 1 PublicationCorresponds to variant dbSNP:rs121917873EnsemblClinVar.	1
Natural variantⁱVAR_002160	331	R → W in HCP. 2 PublicationsCorresponds to variant dbSNP:rs121917866EnsemblClinVar.	1
Natural variantⁱVAR_048827	352	R → C. Corresponds to variant dbSNP:rs11921054EnsemblClinVar.	1
Natural variantⁱVAR_002161	390	Missing in HCP. 1 Publication	1
Natural variantⁱVAR_002162	404	K → E in HCP; harderoporphyria form. 1 PublicationCorresponds to variant dbSNP:rs121917868EnsemblClinVar.	1
Natural variantⁱVAR_002163	427	W → R in HCP. 1 Publication	1
Natural variantⁱVAR_019069	447	R → C in HCP. 1 PublicationCorresponds to variant dbSNP:rs28931603EnsemblClinVar.	1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Alternative sequenceⁱVSP_057182	272 – 287	NKQWW…LTPTY → KGLRSYGKYCRAKCAF in isoform 2. 1 PublicationAdd BLAST		16
Alternative sequenceⁱVSP_057183	288 – 454	Missing in isoform 2. 1 PublicationAdd BLAST		167

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
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS2932.1 [P36551-1]
PIRⁱ	I52444
NCBI Reference Sequences More...RefSeqⁱ	NP_000088.3, NM_000097.5 [P36551-1]
UniGeneⁱ	Hs.476982

Genome annotation databases

Ensemblⁱ	ENST00000264193; ENSP00000264193; ENSG00000080819 [P36551-1]
GeneIDⁱ	1371
KEGGⁱ	hsa:1371
UCSC genome browser More...UCSCⁱ	uc003dsx.4 human [P36551-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P36551	Coproporphyrinogen oxidase		GORGO		464	UniRef90_P36551
CPOX isoform 1		PANTR		454
Uncharacterized protein		RHIRO		454
Uncharacterized protein		RHIBE		454
Uncharacterized protein		Cebus capucinus imitator		453
+24

Protein	Similar proteins		Species	Score	Length	Source
P36551	Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial		RAT		443	UniRef50_P36551
Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial		MOUSE		443
Coproporphyrinogen oxidase		GORGO		464
Uncharacterized protein		RHIBE		454
CPOX isoform 1		PANTR		454
+211

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
CCDSⁱ	CCDS2932.1 [P36551-1]
PIRⁱ	I52444
RefSeqⁱ	NP_000088.3, NM_000097.5 [P36551-1]
UniGeneⁱ	Hs.476982

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
	2AEX	X-ray	1.58	A	111-454	[»]
ProteinModelPortalⁱ	P36551
SMRⁱ	P36551
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	107763, 14 interactors
IntActⁱ	P36551, 21 interactors
MINTⁱ	P36551
STRINGⁱ	9606.ENSP00000264193

Chemistry databases

ChEMBLⁱ	CHEMBL1681618

ChEMBLⁱ

CHEMBL1681618

PTM databases

iPTMnetⁱ	P36551
PhosphoSitePlusⁱ	P36551
SwissPalmⁱ	P36551

Polymorphism and mutation databases

BioMutaⁱ	CPOX
DMDMⁱ	67476671

Proteomic databases

EPDⁱ	P36551
MaxQBⁱ	P36551
PaxDbⁱ	P36551
PeptideAtlasⁱ	P36551
PRIDEⁱ	P36551
ProteomicsDBⁱ	55213

Protocols and materials databases

Structural Biology Knowledgebase	Search...

Structural Biology Knowledgebase

Search...

Genome annotation databases

Ensemblⁱ	ENST00000264193; ENSP00000264193; ENSG00000080819 [P36551-1]
GeneIDⁱ	1371
KEGGⁱ	hsa:1371
UCSCⁱ	uc003dsx.4 human [P36551-1]

Organism-specific databases

CTDⁱ	1371
DisGeNETⁱ	1371
EuPathDBⁱ	HostDB:ENSG00000080819.6
GeneCardsⁱ	CPOX
GeneReviewsⁱ	CPOX
HGNCⁱ	HGNC:2321 CPOX
HPAⁱ	HPA015736 HPA054448
MalaCardsⁱ	CPOX
MIMⁱ	121300 phenotype 612732 gene
neXtProtⁱ	NX_P36551
OpenTargetsⁱ	ENSG00000080819
Orphanetⁱ	79273 Hereditary coproporphyria
PharmGKBⁱ	PA134979958
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG1518 Eukaryota COG0408 LUCA
GeneTreeⁱ	ENSGT00390000017311
HOGENOMⁱ	HOG000262768
HOVERGENⁱ	HBG051897
InParanoidⁱ	P36551
KOⁱ	K00228
OMAⁱ	MDLTPYY
OrthoDBⁱ	EOG091G06ZF
PhylomeDBⁱ	P36551
TreeFamⁱ	TF300703

Enzyme and pathway databases

UniPathwayⁱ	UPA00251;UER00322
BioCycⁱ	MetaCyc:HS01369-MONOMER
BRENDAⁱ	1.3.3.3 2681
Reactomeⁱ	R-HSA-189451 Heme biosynthesis

Miscellaneous databases

ChiTaRSⁱ	CPOX human
EvolutionaryTraceⁱ	P36551
GeneWikiⁱ	Coproporphyrinogen_III_oxidase
GenomeRNAiⁱ	1371
Protein Ontology More...PROⁱ	PR:P36551
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000080819 Expressed in 214 organ(s), highest expression level in liver
CleanExⁱ	HS_CPO HS_CPOX
ExpressionAtlasⁱ	P36551 baseline and differential
Genevisibleⁱ	P36551 HS

Family and domain databases

Gene3Dⁱ	3.40.1500.10, 1 hit
InterProⁱ	View protein in InterPro IPR001260 Coprogen_oxidase_aer IPR036406 Coprogen_oxidase_aer_sf IPR018375 Coprogen_oxidase_CS
PANTHERⁱ	PTHR10755 PTHR10755, 1 hit
Pfamⁱ	View protein in Pfam PF01218 Coprogen_oxidas, 1 hit
PRINTSⁱ	PR00073 COPRGNOXDASE
SUPFAMⁱ	SSF102886 SSF102886, 1 hit
PROSITEⁱ	View protein in PROSITE PS01021 COPROGEN_OXIDASE, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	HEM6_HUMAN
Accessionⁱ	P36551Primary (citable) accession number: P36551 Secondary accession number(s): A8K275 , B4DSD5, Q14060, Q53F08, Q8IZ45, Q96AF3
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	June 1, 1994
	Last sequence update:	June 7, 2005
	Last modified:	November 7, 2018
	This is version 189 of the entry and version 3 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, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human chromosome 3
Human chromosome 3: 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

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - P36551 (HEM6_HUMAN)

Your basket is currently empty. i <p>When browsing through different UniProt proteins, you can use the ‘basket’ to save them, so that you can back to find or analyse them later.<p><a href='/help/basket' target='_top'>More...</a></p>

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

Select a section on the left to see content.

<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

<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

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

Organism-specific databases

<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

Mitochondrion

Cytosol

Mitochondrion

Other locations

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

Mutagenesis

Keywords - Diseasei

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

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

GO - Molecular functioni

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

Keywords - Domaini

Phylogenomic databases

Family and domain databases

<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

Experimental Info

Natural variant

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<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

3D structure databases

Protein-protein interaction databases

Chemistry databases

PTM databases

Polymorphism and mutation 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

Keywords - Technical termi

Your basket is currently empty. ⁱ

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

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Sequence cautionⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ