Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

Gene

Cpox

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

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 (By similarity).By similarity

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:
  1. 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 keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei233SubstrateBy similarity1
Active sitei247Proton donorBy similarity1
Sitei316Important for dimerizationBy similarity1

GO - Molecular functioni

  • coproporphyrinogen oxidase activity Source: MGI
  • protein homodimerization activity Source: UniProtKB
  • sodium:potassium-exchanging ATPase activity Source: MGI
  • structural constituent of eye lens Source: MGI

GO - Biological processi

  • heme biosynthetic process Source: MGI
  • potassium ion transport Source: MGI
  • protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
  • response to arsenic-containing substance Source: Ensembl
  • response to insecticide Source: Ensembl
  • response to iron ion Source: Ensembl
  • response to lead ion Source: Ensembl
  • response to methylmercury Source: Ensembl
  • sodium ion transport Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Heme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

ReactomeiR-MMU-189451. Heme biosynthesis.
UniPathwayiUPA00251; 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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 16

Organism-specific databases

MGIiMGI:104841. Cpox.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: MGI
  • membrane Source: ParkinsonsUK-UCL
  • mitochondrial inner membrane Source: Ensembl
  • mitochondrial intermembrane space Source: UniProtKB-SubCell
  • mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 98Mitochondrion1 PublicationAdd BLAST98
ChainiPRO_000000603099 – 443Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrialAdd BLAST345

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei101PhosphoserineBy similarity1
Modified residuei393N6-acetyllysine; alternateCombined sources1
Modified residuei393N6-succinyllysine; alternateCombined sources1

Post-translational modificationi

Acetylation of Lys-360 is observed in liver mitochondria from fasted mice but not from fed mice.

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP36552.
MaxQBiP36552.
PaxDbiP36552.
PeptideAtlasiP36552.
PRIDEiP36552.

PTM databases

iPTMnetiP36552.
PhosphoSitePlusiP36552.
SwissPalmiP36552.

Expressioni

Tissue specificityi

Erythroid cells and non erythroid cells such as liver.

Gene expression databases

BgeeiENSMUSG00000022742.
CleanExiMM_CPOX.
GenevisibleiP36552. MM.

Interactioni

Subunit structurei

Homodimer.By similarity

GO - Molecular functioni

Protein-protein interaction databases

IntActiP36552. 4 interactors.
MINTiMINT-1844590.
STRINGi10090.ENSMUSP00000055455.

Structurei

3D structure databases

ProteinModelPortaliP36552.
SMRiP36552.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni182 – 191Important for dimerizationBy similarity10
Regioni249 – 251Substrate bindingBy similarity3
Regioni381 – 417Important for dimerizationBy similarityAdd BLAST37
Regioni400 – 405Substrate bindingBy similarity6

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1518. Eukaryota.
COG0408. LUCA.
GeneTreeiENSGT00390000017311.
HOGENOMiHOG000262768.
HOVERGENiHBG051897.
InParanoidiP36552.
KOiK00228.
OMAiMELLIME.
OrthoDBiEOG091G06ZF.
PhylomeDBiP36552.
TreeFamiTF300703.

Family and domain databases

Gene3Di3.40.1500.10. 1 hit.
InterProiIPR001260. Coprogen_oxidase_aer.
IPR018375. Coprogen_oxidase_CS.
[Graphical view]
PANTHERiPTHR10755. PTHR10755. 1 hit.
PfamiPF01218. Coprogen_oxidas. 1 hit.
[Graphical view]
PRINTSiPR00073. COPRGNOXDASE.
SUPFAMiSSF102886. SSF102886. 1 hit.
PROSITEiPS01021. COPROGEN_OXIDASE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

P36552-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MALRLGRLGS DPWWRAVLGD YAQLRAASPR CASARVCQLP GTAGPQPRRG
60 70 80 90 100
LGYGPWARGG SGLGTRLAAT LAGLAGLAAA AFGHVQRAEM VPKSSGARSP
110 120 130 140 150
SPGRREEDGD ELARRCSTFM SSPVTELREL RRRPEDMKTK MELMIMETQA
160 170 180 190 200
QVCRALAQVD GVADFTVDRW ERKEGGGGIT CVLQDGRVFE KAGVSISVVH
210 220 230 240 250
GNLSEEAANQ MRGRGKTLKT KDSKLPFTAM GVSSVIHPKN PYAPTMHFNY
260 270 280 290 300
RYFEVEEADG NTHWWFGGGC DLTPTYLNQE DAVHFHRTLK EACDQHGPDI
310 320 330 340 350
YPKFKKWCDD YFFIVHRGER RGIGGIFFDD LDSPSKEEAF RFVKTCAEAV
360 370 380 390 400
VPSYVPIVKK HCDDSYTPRD KLWQQLRRGR YVEFNLLYDR GTKFGLFTPG
410 420 430 440
SRIESILMSL PLTARWEYMH SPPENSKEAE ILEVLRHPKD WVH
Length:443
Mass (Da):49,715
Last modified:June 21, 2004 - v2
Checksum:i9F3D5E8E420645F0
GO

Sequence cautioni

The sequence AAH17680 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence BAA03840 differs from that shown. Reason: Frameshift at positions 22, 43 and 76.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti7R → P in BAA03840 (PubMed:8407975).Curated1
Sequence conflicti101S → T AA sequence (PubMed:8159699).Curated1
Sequence conflicti116 – 117CS → SD AA sequence (PubMed:8159699).Curated2
Sequence conflicti122S → T AA sequence (PubMed:8159699).Curated1
Sequence conflicti124V → P AA sequence (PubMed:8159699).Curated1
Sequence conflicti138K → N in BAC79229 (PubMed:15482256).Curated1
Sequence conflicti275T → R in BAA03840 (PubMed:8407975).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D16333 mRNA. Translation: BAA03840.1. Frameshift.
BC017680 mRNA. Translation: AAH17680.2. Different initiation.
AY382578 Genomic DNA. Translation: AAQ88103.1.
AB099924 Genomic DNA. Translation: BAC79229.1.
CCDSiCCDS49878.1.
PIRiA48049.
RefSeqiNP_031783.2. NM_007757.2.
UniGeneiMm.291519.

Genome annotation databases

EnsembliENSMUST00000060077; ENSMUSP00000055455; ENSMUSG00000022742.
GeneIDi12892.
KEGGimmu:12892.
UCSCiuc007znz.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D16333 mRNA. Translation: BAA03840.1. Frameshift.
BC017680 mRNA. Translation: AAH17680.2. Different initiation.
AY382578 Genomic DNA. Translation: AAQ88103.1.
AB099924 Genomic DNA. Translation: BAC79229.1.
CCDSiCCDS49878.1.
PIRiA48049.
RefSeqiNP_031783.2. NM_007757.2.
UniGeneiMm.291519.

3D structure databases

ProteinModelPortaliP36552.
SMRiP36552.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP36552. 4 interactors.
MINTiMINT-1844590.
STRINGi10090.ENSMUSP00000055455.

PTM databases

iPTMnetiP36552.
PhosphoSitePlusiP36552.
SwissPalmiP36552.

Proteomic databases

EPDiP36552.
MaxQBiP36552.
PaxDbiP36552.
PeptideAtlasiP36552.
PRIDEiP36552.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000060077; ENSMUSP00000055455; ENSMUSG00000022742.
GeneIDi12892.
KEGGimmu:12892.
UCSCiuc007znz.2. mouse.

Organism-specific databases

CTDi1371.
MGIiMGI:104841. Cpox.

Phylogenomic databases

eggNOGiKOG1518. Eukaryota.
COG0408. LUCA.
GeneTreeiENSGT00390000017311.
HOGENOMiHOG000262768.
HOVERGENiHBG051897.
InParanoidiP36552.
KOiK00228.
OMAiMELLIME.
OrthoDBiEOG091G06ZF.
PhylomeDBiP36552.
TreeFamiTF300703.

Enzyme and pathway databases

UniPathwayiUPA00251; UER00322.
ReactomeiR-MMU-189451. Heme biosynthesis.

Miscellaneous databases

ChiTaRSiCpox. mouse.
PROiP36552.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000022742.
CleanExiMM_CPOX.
GenevisibleiP36552. MM.

Family and domain databases

Gene3Di3.40.1500.10. 1 hit.
InterProiIPR001260. Coprogen_oxidase_aer.
IPR018375. Coprogen_oxidase_CS.
[Graphical view]
PANTHERiPTHR10755. PTHR10755. 1 hit.
PfamiPF01218. Coprogen_oxidas. 1 hit.
[Graphical view]
PRINTSiPR00073. COPRGNOXDASE.
SUPFAMiSSF102886. SSF102886. 1 hit.
PROSITEiPS01021. COPROGEN_OXIDASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiHEM6_MOUSE
AccessioniPrimary (citable) accession number: P36552
Secondary accession number(s): Q7TQ36, Q8VD08
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: June 21, 2004
Last modified: November 2, 2016
This is version 147 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.