UniProt Accession

 HEM6_HUMAN; P36551; A8K275; Q14060; Q53F08; Q8IZ45; Q96AF3 

Genbank Protein ID

 Z34531; Z34803; Z34804; Z34805; Z34806; Z34807; Z34808; AK290140; AK223481; CH471052; BC017210; BC023551; BC023554; D16611; Z28409 

Genbank Nucleotide ID

 CAA84292.1; CAA84292.1; CAA84292.1; CAA84292.1; CAA84292.1; CAA84292.1; CAA84292.1; BAF82829.1; BAD97201.1; EAW79854.1; AAH17210.1; AAH23551.1; AAH23554.1; BAA04033.1; CAA82250.1 

Protein Name

 Coproporphyrinogen-III oxidase, mitochondrial 

Protein Synonyms/Alias

 COX; Coprogen oxidase; Coproporphyrinogenase 

Gene Name

 CPOX 

Gene Synonyms/Alias

 CPO; CPX 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
184	SVDRWERKEGGGGIS	ubiquitination	[1]
220	NLSEEAAKQMRSRGK	ubiquitination	[1, 2, 3]
232	RGKVLKTKDGKLPFC	ubiquitination	[1]
235	VLKTKDGKLPFCAMG	ubiquitination	[1]
347	DDLDSPSKEEVFRFV	ubiquitination	[1, 2, 3]
370	PSYIPLVKKHCDDSF	ubiquitination	[2, 3]
371	SYIPLVKKHCDDSFT	ubiquitination	[1]
404	LLYDRGTKFGLFTPG	ubiquitination	[2, 3]
438	HSPSENSKEAEILEV	ubiquitination	[2]

Reference

 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [2] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [3] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661] 

Functional Description

 Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. 

Sequence Annotation

 REGION 193 202 Important for dimerization.
 REGION 260 262 Substrate binding.
 REGION 392 428 Important for dimerization.
 REGION 411 416 Substrate binding.
 ACT_SITE 258 258 Proton donor.
 BINDING 244 244 Substrate.
 MOD_RES 371 371 N6-acetyllysine (By similarity).  

Keyword

 3D-structure; Acetylation; Complete proteome; Disease mutation; Heme biosynthesis; Mitochondrion; Oxidoreductase; Polymorphism; Porphyrin biosynthesis; Reference proteome; Transit peptide. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 454 AA 

Protein Sequence

Gene Ontology

 GO:0005743; C:mitochondrial inner membrane; IEA:Compara.
 GO:0005758; C:mitochondrial intermembrane space; TAS:Reactome.
 GO:0004109; F:coproporphyrinogen oxidase activity; TAS:Reactome.
 GO:0042803; F:protein homodimerization activity; IDA:UniProtKB.
 GO:0006783; P:heme biosynthetic process; TAS:Reactome.
 GO:0006782; P:protoporphyrinogen IX biosynthetic process; IEA:UniProtKB-UniPathway.
 GO:0046685; P:response to arsenic-containing substance; IEA:Compara.
 GO:0017085; P:response to insecticide; IEA:Compara.
 GO:0010039; P:response to iron ion; IEA:Compara.
 GO:0010288; P:response to lead ion; IEA:Compara.
 GO:0051597; P:response to methylmercury; IEA:Compara.
 GO:0044281; P:small molecule metabolic process; TAS:Reactome. 

Interpro

 IPR001260; Coprogen_oxidase_aer.
 IPR018375; Coprogen_oxidase_CS. 

Pfam

 PF01218; Coprogen_oxidas 

SMART

  

PROSITE

 PS01021; COPROGEN_OXIDASE 

PRINTS

 PR00073; COPRGNOXDASE.