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Protein

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

Gene

Cpox

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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
  • identical protein binding Source: MGI
  • protein homodimerization activity Source: UniProtKB
  • sodium:potassium-exchanging ATPase activity Source: MGI
  • structural constituent of eye lens Source: MGI

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
Biological processHeme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

ReactomeiR-MMU-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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 16

Organism-specific databases

MGIiMGI:104841 Cpox

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

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 Expressed in 280 organ(s), highest expression level in liver
CleanExiMM_CPOX
GenevisibleiP36552 MM

Interactioni

Subunit structurei

Homodimer.By similarity

GO - Molecular functioni

Protein-protein interaction databases

IntActiP36552, 4 interactors
MINTiP36552
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
OMAiMDLTPYY
OrthoDBiEOG091G06ZF
PhylomeDBiP36552
TreeFamiTF300703

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

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

Similar proteinsi

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
MINTiP36552
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
OMAiMDLTPYY
OrthoDBiEOG091G06ZF
PhylomeDBiP36552
TreeFamiTF300703

Enzyme and pathway databases

UniPathwayi
UPA00251;UER00322

ReactomeiR-MMU-189451 Heme biosynthesis

Miscellaneous databases

ChiTaRSiCpox mouse
PROiPR:P36552
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000022742 Expressed in 280 organ(s), highest expression level in liver
CleanExiMM_CPOX
GenevisibleiP36552 MM

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
ProtoNetiSearch...

Entry informationi

Entry nameiHEM6_MOUSE
AccessioniPrimary (citable) accession number: P36552
Secondary accession number(s): Q7TQ36, Q8VD08
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: June 21, 2004
Last modified: November 7, 2018
This is version 156 of the entry and version 2 of the sequence. See 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. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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