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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

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei233 – 2331SubstrateBy similarity
Active sitei247 – 2471Proton donorBy similarity
Sitei316 – 3161Important for dimerizationBy similarity

GO - Molecular functioni

  1. coproporphyrinogen oxidase activity Source: MGI
  2. protein homodimerization activity Source: UniProtKB
  3. sodium:potassium-exchanging ATPase activity Source: MGI
  4. structural constituent of eye lens Source: MGI

GO - Biological processi

  1. heme biosynthetic process Source: MGI
  2. potassium ion transport Source: MGI
  3. protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
  4. response to arsenic-containing substance Source: Ensembl
  5. response to insecticide Source: Ensembl
  6. response to iron ion Source: Ensembl
  7. response to lead ion Source: Ensembl
  8. response to methylmercury Source: Ensembl
  9. sodium ion transmembrane transport Source: GOC
  10. sodium ion transport Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Heme biosynthesis, Porphyrin biosynthesis

Enzyme and pathway databases

ReactomeiREACT_203298. 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
ProteomesiUP000000589: Chromosome 16

Organism-specific databases

MGIiMGI:104841. Cpox.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: Ensembl
  2. mitochondrial intermembrane space Source: UniProtKB-SubCell
  3. mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 9898Mitochondrion1 PublicationAdd
BLAST
Chaini99 – 443345Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrialPRO_0000006030Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei393 – 3931N6-acetyllysine; alternate1 Publication
Modified residuei393 – 3931N6-succinyllysine; alternate1 Publication

Post-translational modificationi

Acetylation of Lys-360 is observed in liver mitochondria from fasted mice but not from fed mice.1 Publication

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiP36552.
PaxDbiP36552.
PRIDEiP36552.

PTM databases

PhosphoSiteiP36552.

Expressioni

Tissue specificityi

Erythroid cells and non erythroid cells such as liver.

Gene expression databases

BgeeiP36552.
CleanExiMM_CPOX.
ExpressionAtlasiP36552. baseline and differential.
GenevestigatoriP36552.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

IntActiP36552. 1 interaction.
MINTiMINT-1844590.

Structurei

3D structure databases

ProteinModelPortaliP36552.
SMRiP36552. Positions 110-442.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni182 – 19110Important for dimerizationBy similarity
Regioni249 – 2513Substrate bindingBy similarity
Regioni381 – 41737Important for dimerizationBy similarityAdd
BLAST
Regioni400 – 4056Substrate bindingBy similarity

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0408.
GeneTreeiENSGT00390000017311.
HOGENOMiHOG000262768.
HOVERGENiHBG051897.
InParanoidiP36552.
KOiK00228.
OMAiQRPEAKG.
OrthoDBiEOG7BZVSG.
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 AAH17680.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence BAA03840.1 differs from that shown. Reason: Frameshift at positions 22, 43 and 76. Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti7 – 71R → P in BAA03840. (PubMed:8407975)Curated
Sequence conflicti101 – 1011S → T AA sequence (PubMed:8159699)Curated
Sequence conflicti116 – 1172CS → SD AA sequence (PubMed:8159699)Curated
Sequence conflicti122 – 1221S → T AA sequence (PubMed:8159699)Curated
Sequence conflicti124 – 1241V → P AA sequence (PubMed:8159699)Curated
Sequence conflicti138 – 1381K → N in BAC79229. (PubMed:15482256)Curated
Sequence conflicti275 – 2751T → R in BAA03840. (PubMed:8407975)Curated

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. Positions 110-442.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP36552. 1 interaction.
MINTiMINT-1844590.

PTM databases

PhosphoSiteiP36552.

Proteomic databases

MaxQBiP36552.
PaxDbiP36552.
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

eggNOGiCOG0408.
GeneTreeiENSGT00390000017311.
HOGENOMiHOG000262768.
HOVERGENiHBG051897.
InParanoidiP36552.
KOiK00228.
OMAiQRPEAKG.
OrthoDBiEOG7BZVSG.
PhylomeDBiP36552.
TreeFamiTF300703.

Enzyme and pathway databases

UniPathwayiUPA00251; UER00322.
ReactomeiREACT_203298. Heme biosynthesis.

Miscellaneous databases

ChiTaRSiCpox. mouse.
NextBioi282498.
PROiP36552.
SOURCEiSearch...

Gene expression databases

BgeeiP36552.
CleanExiMM_CPOX.
ExpressionAtlasiP36552. baseline and differential.
GenevestigatoriP36552.

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...

Publicationsi

« Hide 'large scale' publications
  1. "Coproporphyrinogen oxidase. Purification, molecular cloning, and induction of mRNA during erythroid differentiation."
    Kohno H., Furukawa T., Yoshinaga T., Tokunaga R., Taketani S.
    J. Biol. Chem. 268:21359-21363(1993) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Kidney.
  3. "Examination of mitochondrial protein targeting of haem synthetic enzymes: in vivo identification of three functional haem-responsive motifs in 5-aminolaevulinate synthase."
    Dailey T.A., Woodruff J.H., Dailey H.A.
    Biochem. J. 386:381-386(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-139.
  4. "The long, but not the short, presequence of human coproporphyrinogen oxidase is essential for its import and sorting to mitochondria."
    Susa S., Daimon M., Ono H., Li S., Yoshida T., Kato T.
    Tohoku J. Exp. Med. 200:39-45(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-144.
  5. "Molecular cloning, sequencing, and functional expression of a cDNA encoding human coproporphyrinogen oxidase."
    Martasek P., Camadro J.-M., Delfau-Larue M.H., Dumas J.B., Montagne J.J., de Verneuil H., Labbe P., Grandchamp B.
    Proc. Natl. Acad. Sci. U.S.A. 91:3024-3028(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 99-125 AND 248-258.
  6. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
    Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
    Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-393, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  7. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
    Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
    Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-393, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

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: January 7, 2015
This is version 133 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

External Data

Dasty 3

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 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.