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P36552 (HEM6_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 128. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

Short name=COX
Short name=Coprogen oxidase
Short name=Coproporphyrinogenase
EC=1.3.3.3
Gene names
Name:Cpox
Synonyms:Cpo
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length443 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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.

Catalytic activity

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

Pathway

Porphyrin-containing compound metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.

Subunit structure

Homodimer By similarity.

Subcellular location

Mitochondrion intermembrane space.

Tissue specificity

Erythroid cells and non erythroid cells such as liver.

Post-translational modification

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

Sequence similarities

Belongs to the aerobic coproporphyrinogen-III oxidase family.

Sequence caution

The sequence AAH17680.2 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.

The sequence BAA03840.1 differs from that shown. Reason: Frameshift at positions 22, 43 and 76.

Ontologies

Keywords
   Biological processHeme biosynthesis
Porphyrin biosynthesis
   Cellular componentMitochondrion
   DomainTransit peptide
   Molecular functionOxidoreductase
   PTMAcetylation
   Technical termComplete proteome
Direct protein sequencing
Reference proteome
Gene Ontology (GO)
   Biological_processheme biosynthetic process

Inferred from direct assay Ref.1. Source: MGI

potassium ion transport

Traceable author statement PubMed 12470976. Source: MGI

protoporphyrinogen IX biosynthetic process

Inferred from electronic annotation. Source: UniProtKB-UniPathway

response to arsenic-containing substance

Inferred from electronic annotation. Source: Ensembl

response to insecticide

Inferred from electronic annotation. Source: Ensembl

response to iron ion

Inferred from electronic annotation. Source: Ensembl

response to lead ion

Inferred from electronic annotation. Source: Ensembl

response to methylmercury

Inferred from electronic annotation. Source: Ensembl

sodium ion transmembrane transport

Traceable author statement PubMed 12470976. Source: GOC

sodium ion transport

Traceable author statement PubMed 12470976. Source: MGI

   Cellular_componentmitochondrial inner membrane

Inferred from electronic annotation. Source: Ensembl

mitochondrial intermembrane space

Inferred from electronic annotation. Source: UniProtKB-SubCell

mitochondrion

Inferred from direct assay PubMed 14651853Ref.3PubMed 18614015Ref.1. Source: MGI

   Molecular_functioncoproporphyrinogen oxidase activity

Inferred from direct assay Ref.1. Source: MGI

protein homodimerization activity

Inferred from sequence or structural similarity. Source: UniProtKB

sodium:potassium-exchanging ATPase activity

Traceable author statement PubMed 12470976. Source: MGI

structural constituent of eye lens

Inferred from mutant phenotype PubMed 12470976. Source: MGI

Complete GO annotation...

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 9898Mitochondrion Ref.5
Chain99 – 443345Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
PRO_0000006030

Regions

Region182 – 19110Important for dimerization By similarity
Region249 – 2513Substrate binding By similarity
Region381 – 41737Important for dimerization By similarity
Region400 – 4056Substrate binding By similarity

Sites

Active site2471Proton donor By similarity
Binding site2331Substrate By similarity
Site3161Important for dimerization By similarity

Amino acid modifications

Modified residue3931N6-acetyllysine; alternate Ref.7
Modified residue3931N6-succinyllysine; alternate Ref.6

Experimental info

Sequence conflict71R → P in BAA03840. Ref.1
Sequence conflict1011S → T AA sequence Ref.5
Sequence conflict116 – 1172CS → SD AA sequence Ref.5
Sequence conflict1221S → T AA sequence Ref.5
Sequence conflict1241V → P AA sequence Ref.5
Sequence conflict1381K → N in BAC79229. Ref.3
Sequence conflict2751T → R in BAA03840. Ref.1

Sequences

Sequence LengthMass (Da)Tools
P36552 [UniParc].

Last modified June 21, 2004. Version 2.
Checksum: 9F3D5E8E420645F0

FASTA44349,715
        10         20         30         40         50         60 
MALRLGRLGS DPWWRAVLGD YAQLRAASPR CASARVCQLP GTAGPQPRRG LGYGPWARGG 

        70         80         90        100        110        120 
SGLGTRLAAT LAGLAGLAAA AFGHVQRAEM VPKSSGARSP SPGRREEDGD ELARRCSTFM 

       130        140        150        160        170        180 
SSPVTELREL RRRPEDMKTK MELMIMETQA QVCRALAQVD GVADFTVDRW ERKEGGGGIT 

       190        200        210        220        230        240 
CVLQDGRVFE KAGVSISVVH GNLSEEAANQ MRGRGKTLKT KDSKLPFTAM GVSSVIHPKN 

       250        260        270        280        290        300 
PYAPTMHFNY RYFEVEEADG NTHWWFGGGC DLTPTYLNQE DAVHFHRTLK EACDQHGPDI 

       310        320        330        340        350        360 
YPKFKKWCDD YFFIVHRGER RGIGGIFFDD LDSPSKEEAF RFVKTCAEAV VPSYVPIVKK 

       370        380        390        400        410        420 
HCDDSYTPRD KLWQQLRRGR YVEFNLLYDR GTKFGLFTPG SRIESILMSL PLTARWEYMH 

       430        440 
SPPENSKEAE ILEVLRHPKD WVH 

« Hide

References

« Hide 'large scale' references
[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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
CCDSCCDS49878.1.
PIRA48049.
RefSeqNP_031783.2. NM_007757.2.
UniGeneMm.291519.

3D structure databases

ProteinModelPortalP36552.
SMRP36552. Positions 110-442.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

IntActP36552. 1 interaction.
MINTMINT-1844590.

PTM databases

PhosphoSiteP36552.

Proteomic databases

MaxQBP36552.
PaxDbP36552.
PRIDEP36552.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000060077; ENSMUSP00000055455; ENSMUSG00000022742.
GeneID12892.
KEGGmmu:12892.
UCSCuc007znz.2. mouse.

Organism-specific databases

CTD1371.
MGIMGI:104841. Cpox.

Phylogenomic databases

eggNOGCOG0408.
GeneTreeENSGT00390000017311.
HOGENOMHOG000262768.
HOVERGENHBG051897.
InParanoidP36552.
KOK00228.
OMARYFETAN.
OrthoDBEOG7BZVSG.
PhylomeDBP36552.
TreeFamTF300703.

Enzyme and pathway databases

UniPathwayUPA00251; UER00322.

Gene expression databases

ArrayExpressP36552.
BgeeP36552.
CleanExMM_CPOX.
GenevestigatorP36552.

Family and domain databases

Gene3D3.40.1500.10. 1 hit.
InterProIPR001260. Coprogen_oxidase_aer.
IPR018375. Coprogen_oxidase_CS.
[Graphical view]
PANTHERPTHR10755. PTHR10755. 1 hit.
PfamPF01218. Coprogen_oxidas. 1 hit.
[Graphical view]
PRINTSPR00073. COPRGNOXDASE.
SUPFAMSSF102886. SSF102886. 1 hit.
PROSITEPS01021. COPROGEN_OXIDASE. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

ChiTaRSCPOX. mouse.
NextBio282498.
PROP36552.
SOURCESearch...

Entry information

Entry nameHEM6_MOUSE
AccessionPrimary (citable) accession number: P36552
Secondary accession number(s): Q7TQ36, Q8VD08
Entry history
Integrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: June 21, 2004
Last modified: July 9, 2014
This is version 128 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

PATHWAY comments

Index of metabolic and biosynthesis pathways

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot