Reviewed, UniProtKB/Swiss-Prot P36552 (HEM6_MOUSE)

Names and origin

Protein names

Recommended name:
    Coproporphyrinogen-III oxidase, mitochondrial
      Short name=Coproporphyrinogenase
      Short name=Coprogen oxidase
      Short name=COX
    EC=1.3.3.3

Gene names

Name:	Cpox
Synonyms:	Cpo

Organism

Mus musculus (Mouse)

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus

Protein attributes

Sequence length	443 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level.

General annotation (Comments)

Catalytic activity	Coproporphyrinogen-III + O2 + 2 H+ = protoporphyrinogen-IX + 2 CO2 + 2 H2O.
Pathway	Porphyrin metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.
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 BAA03840.1 differs from that shown. Reason: Frameshift at positions 22, 43 and 76.

Ontologies

Keywords
Biological process	Heme biosynthesis Porphyrin biosynthesis
Cellular component	Mitochondrion
Domain	Transit peptide
Molecular function	Oxidoreductase
PTM	Acetylation Phosphoprotein
Technical term	Direct protein sequencing
Gene Ontology (GO)
Biological process	heme biosynthetic process Ref.1 Inferred from direct assay. Source: MGI oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	mitochondrial intermembrane space Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	coproporphyrinogen oxidase activity Ref.1 Inferred from direct assay. Source: MGI
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 98	98	Mitochondrion Ref.5
	Chain	99 – 443	345	Coproporphyrinogen-III oxidase, mitochondrial		PRO_0000006030
Amino acid modifications
	Modified residue	99	1	Phosphoserine Ref.7
	Modified residue	360	1	N6-acetyllysine Ref.6
Experimental info
	Sequence conflict	7	1	R → P in BAA03840. Ref.1
	Sequence conflict	101	1	S → T AA sequence Ref.5
	Sequence conflict	116 – 117	2	CS → SD AA sequence Ref.5
	Sequence conflict	122	1	S → T AA sequence Ref.5
	Sequence conflict	124	1	V → P AA sequence Ref.5
	Sequence conflict	138	1	K → N in BAC79229. Ref.3
	Sequence conflict	275	1	T → R in BAA03840. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	P36552-1 [UniParc]. Last modified June 21, 2004. Version 2. Checksum: 9F3D5E8E420645F0	FASTA	443	49,715
	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

References

	« Hide 'large scale' referencesShow '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: 8407975] [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: 15489334] [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: 15482256] [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: 12862310] [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: 8159699] [Abstract] Cited for: PROTEIN SEQUENCE OF 99-125 AND 248-258.
[6]	"Substrate and functional diversity of lysine acetylation revealed by a proteomics survey." Kim S.C., Sprung R., Chen Y., Xu Y., Ball H., Pei J., Cheng T., Kho Y., Xiao H., Xiao L., Grishin N.V., White M., Yang X.-J., Zhao Y. Mol. Cell 23:607-618(2006) [PubMed: 16916647] [Abstract] Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-360, MASS SPECTROMETRY. Tissue: Liver.
[7]	"Large-scale phosphorylation analysis of mouse liver." Villen J., Beausoleil S.A., Gerber S.A., Gygi S.P. Proc. Natl. Acad. Sci. U.S.A. 104:1488-1493(2007) [PubMed: 17242355] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-99, MASS SPECTROMETRY. Tissue: Liver.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBLGenBankDDBJ	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.
IPI	IPI00400301.
PIR	A48049.
RefSeq	NP_031783.2.
UniGene	Mm.291519
3D structure databases
SMR	P36552. Positions 110-442.
ModBase	Search...
PTM databases
PhosphoSite	P36552.
Genome annotation databases
Ensembl	ENSMUSG00000022742. Mus musculus. [Contig view]
GeneID	12892.
KEGG	mmu:12892.
NMPDR	fig|10090.3.peg.31449.
Organism-specific databases
MGI	MGI:104841. Cpox.
Phylogenomic databases
HOGENOM	P36552.
HOVERGEN	P36552.
OMA	P36552. VFKPWCD.
Enzyme and pathway databases
BRENDA	1.3.3.3. 244.
Gene expression databases
ArrayExpress	P36552.
Bgee	P36552.
CleanEx	MM_CPOX.
GermOnline	ENSMUSG00000022742. Mus musculus.
Family and domain databases
InterPro	IPR001260. Coprogen_oxidas. IPR018375. Coproporphyrinogen-3_ox_CS. [Graphical view]
Gene3D	G3DSA:3.40.1500.10. Coprogen_oxidas. 1 hit.
PANTHER	PTHR10755. Coprogen_oxidas. 1 hit.
Pfam	PF01218. Coprogen_oxidas. 1 hit. [Graphical view]
PRINTS	PR00073. COPRGNOXDASE.
PROSITE	PS01021. COPROGEN_OXIDASE. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	282498.
SOURCE	Search...

Entry information

Entry name

HEM6_MOUSE

Accession

Primary (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:	June 16, 2009
This is version 84 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)