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Category Symbol Source: HGNC EntrezGene Ensembl GeneCards RNA genes CroW21

GIFtS
-

CPOX Gene

protein-coding GIFtS: 61
GCID: GC03M098239

coproporphyrinogen oxidase

(Previous names: coproporphyrinogen oxidase (coproporphyria, harderoporphyria)...)
(Previous symbol: CPO)

Explore 69 diseases affiliated with
CPOX via

MalaCards

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(According to ¹HGNC, ²Entrez Gene,
³UniProtKB/Swiss-Prot, ⁴UniProtKB/TrEMBL, ⁵OMIM, ⁶GeneLoc, ⁷Ensembl, ⁸DME, ⁹miRBase, and/or ¹⁰fRNAdb)
About This Section

Aliases
Coproporphyrinogen Oxidase¹ ²		EC 1.3.3.3³ ⁸
CPO¹ ² ³		Coproporphyrinogen Oxidase (Coproporphyria, Harderoporphyria)¹
CPX¹ ² ³		Coproporphyrinogen-III Oxidase, Mitochondrial²
HCP¹ ²		Coproporphyrinogenase³
Coprogen Oxidase² ³		Coproporphyrinogenase³
COX² ³

External Ids:	HGNC: 2321¹	Entrez Gene: 1371²	Ensembl: ENSG00000080819⁷	OMIM: 612732⁵	UniProtKB: P36551³

Export aliases for CPOX gene to outside databases

Previous GC identifers: GC03M099619 GC03M099780 GC03M098298 GC03M095670

(According to Entrez Gene, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
About This Section

Entrez Gene summary for CPOX:

The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble

and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of

coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary

coproporphyria (HCP).(provided by RefSeq, Oct 2009)

UniProtKB/Swiss-Prot: HEM6_HUMAN, P36551

Function: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of

rings A and B of coproporphyrinogen III

Gene Wiki entry for CPOX (Coproporphyrinogen III oxidase)

(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 69), Regulatory elements and Epigenetics data according to QIAGEN, SABiosciences, and/or SwitchGear Genomics)
About This Section

RefSeq DNA sequence:

NC_000003.11 NC_018914.1 NT_005612.16

Regulatory elements:

SABiosciences Regulatory transcription factor binding sites in the CPOX gene promoter:
HFH-3 GATA-3 YY1 HEN1 AREB6 FOXI1 Max Msx-1 Pax-4a c-Myc
Other transcription factors

SwitchGear Promoter luciferase reporter plasmid: CPOX promoter sequence

Search SABiosciences Chromatin IP Primers for CPOX

Epigenetics:

QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat CPOX

Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: 3q12 Ensembl cytogenetic band: 3q12.1 HGNC cytogenetic band: 3q12

CPOX Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
CPOX gene location

GeneLoc information about chromosome 3 GeneLoc Exon Structure

GeneLoc location for GC03M098239: view genomic region (about GC identifiers)

Start:	98,239,976 bp from pter	End:	98,312,567 bp from pter
Size:	72,592 bases	Orientation:	minus strand

(According to ¹UniProtKB, HORDE, neXtProt, Ensembl, and/or Reactome, Modification sites according to ²PhosphoSitePlus, Specific Peptides from DME, Protein expression images according to data from SPIRE MOPED and PaxDb, RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, and/or Uscn,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, and/or Uscn, Ontologies according to Gene Ontology Consortium 01 Mar 2013 and Entrez Gene, Antibodies by EMD Millipore, R&D Systems, GenScript, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, Abcam, and/or Uscn)
About This Section

UniProtKB/Swiss-Prot: HEM6_HUMAN, P36551 (See protein sequence)

Recommended Name: Coproporphyrinogen-III oxidase, mitochondrial precursor

Size: 454 amino acids; 50152 Da

Subunit: Homodimer

Subcellular location: Mitochondrion intermembrane space

Sequence caution: Sequence=BAA04033.1; Type=Erroneous initiation;

1 PDB 3D structure from and Proteopedia for CPOX:

2AEX (3D)

Secondary accessions: A8K275 Q14060 Q53F08 Q8IZ45 Q96AF3

Explore the universe of human proteins at neXtProt for CPOX: NX_P36551

Post-translational modifications:

View modification sites using PhosphoSitePlus²

View neXtProt modification sites for NX_P36551

4/10 DME Specific Peptides for CPOX (P36551) (see all 10)

WCDDYFF

RGIGGIF

ARWEYMH

RYVEFNL

CPOX Protein expression data from MOPED and PaxDb: About this image

REFSEQ proteins: NP_000088.3

ENSEMBL proteins:

ENSP00000425880 ENSP00000264193 ENSP00000424924

Reactome Protein details: P36551
Human Recombinant Protein Products:

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	Uscn Proteins for CPOX

Gene Ontology (GO): 4 cellular component terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0005737	cytoplasm	IDA	--
GO:0005739	mitochondrion	IDA	--
GO:0005743	mitochondrial inner membrane	IEA	--
GO:0005758	mitochondrial intermembrane space	TAS	--

CPOX for ontologies About GeneDecksing

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	Uscn ELISAs and CLIAs for CPOX

(According to InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
About This Section

CPOX for domains About GeneDecksing

2 InterPro domains/families:

IPR001260 Coprogen_oxidase_aer

IPR018375 Coprogen_oxidase_CS

Graphical View of Domain Structure for InterPro Entry P36551

ProtoNet protein and cluster: P36551

1 Blocks protein family: IPB001260 Coproporphyrinogen III oxidase

UniProtKB/Swiss-Prot: HEM6_HUMAN, P36551

Similarity: Belongs to the aerobic coproporphyrinogen-III oxidase family

(According to ¹UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or ²DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013,
bound targets from SABiosciences, miRNA Gene Targets from miRTarBase shRNA from OriGene, RNAi from EMD Millipore, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, Gene Editing from DNA2.0, Clones from EMD Millipore, OriGene, SwitchGear Genomics, GenScript, Sino Biological, DNA2.0, and Vector BioLabs, Cell Lines from GenScript, LifeMap BioReagents, In Situ Hybridization Assays from Advanced Cell Diagnostics, Ontologies according to Gene Ontology Consortium 01 Mar 2013 via Entrez Gene.)
About This Section

Function Summary:

UniProtKB/Swiss-Prot: HEM6_HUMAN, P36551

Function: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of

rings A and B of coproporphyrinogen III

Catalytic activity: Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O

Enzyme Number (IUBMB): EC 1.3.3.3¹ ²

miRNA Products:		miRTarBase miRNAs that target CPOX: hsa-mir-1 (MIRT001374)

		OriGene 3'-UTR Clone: CPOX Browse MicroRNA Expression Plasmids
		QIAGEN Custom miScript Target Protector blocks miRNA-binding site of human, mouse, rat CPOX
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		hsa-miR-3163 hsa-miR-361-5p hsa-miR-130a* hsa-miR-3653 hsa-miR-216b hsa-miR-23c hsa-miR-4282 hsa-miR-890

		SwitchGear 3'UTR luciferase reporter plasmid: CPOX 3' UTR sequence

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Gene Editing
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In Situ Assay
Products:

Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for CPOX

Gene Ontology (GO): 3 molecular function terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0004109	coproporphyrinogen oxidase activity	TAS	--
GO:0042802	identical protein binding	--	--
GO:0042803	protein homodimerization activity	IDA	16176984

CPOX for ontologies About GeneDecksing

3 GenomeRNAi human phenotypes for CPOX:

Cell cycle / mitosis defect

Increased S DNA content

Increased number of mitotic ce

(Pathways according to EMD Millipore, R&D Systems, Cell Signaling Technology, KEGG, PharmGKB, BioSystems, Reactome, Tocris Bioscience, GeneGo (Thomson Reuters), QIAGEN, and/or UniProtKB, Sets of similar genes according to GeneDecks, Interaction Networks according to SABiosciences, and/or STRING, Interactions according to ¹UniProtKB, ²MINT, ³I2D, and/or ⁴STRING, with links to IntAct and Ensembl, Ontologies according to Gene Ontology Consortium 01 Mar 2013 via Entrez Gene).
About This Section

Unified GeneCards pathways About this table

See pathways by source

Super-pathway

contained gene-specific pathways

heme biosynthesis II

heme biosynthesis II	1.00	Metabolism of porphyrins	0.50
Heme Biosynthesis	0.78	heme biosynthesis from uroporphyrinogen-III I	0.43
Heme biosynthesis	0.78

Metabolism

Metabolism

1.00

Metabolic pathways

0.38

Glucuronidation

Porphyrin and chlorophyll metabolism

0.38

Pathway sources
See GeneCards unified pathways
Show all pathways

3 BioSystems Pathways for CPOX

	Heme Biosynthesis
	heme biosynthesis from uroporphyrinogen-III I
	heme biosynthesis II

3 Reactome Pathways for CPOX

	Metabolism of porphyrins
	Heme biosynthesis
	Metabolism

2 Kegg Pathways (Kegg details for CPOX):

	Porphyrin and chlorophyll metabolism
	Metabolic pathways

UniProtKB/Swiss-Prot: HEM6_HUMAN, P36551

Pathway: Porphyrin metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2

route): step 1/1

CPOX for pathways About GeneDecksing

Interactions:

Search SABiosciences Gene Network Central^TM Interacting Genes and Proteins Networks for CPOX

STRING Interaction Network Preview (showing 5 interactants - click image to see 25)

5/66 Interacting proteins for CPOX (P36551³ ENSP00000264193⁴) via UniProtKB, MINT, STRING, and/or I2D (see all 66)

Interactant		Interaction Details
GeneCard	External ID(s)	Interaction Details
HIST1H4A	P62805³	I2D: score=3
HIST1H4B	P62805³	I2D: score=3
HIST1H4C	P62805³	I2D: score=3
HIST1H4D	P62805³	I2D: score=3
HIST1H4E	P62805³	I2D: score=3

About this table

Gene Ontology (GO): 5/11 biological process terms (GO ID links to tree view) (see all 11): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0006778	porphyrin-containing compound metabolic process	TAS	--
GO:0006779	porphyrin-containing compound biosynthetic process	--	--
GO:0006782	protoporphyrinogen IX biosynthetic process	IEA	--
GO:0006783	heme biosynthetic process	TAS	--
GO:0010035	response to inorganic substance	--	--

CPOX for ontologies About GeneDecksing

(Chemical Compounds according to UniProtKB, Enzo Life Sciences, EMD Millipore, Tocris Bioscience HMDB, BitterDB, and/or Novoseek, and Drugs according to DrugBank, Enzo Life Sciences, and/or PharmGKB, with drugs/clinical trials/news search links to CenterWatch)
About This Section

CPOX for compounds About GeneDecksing

Browse Small Molecules at EMD Millipore

Browse drugs & compounds from Enzo Life Sciences

Browse Tocris compounds for CPOX

9 HMDB Compounds for CPOX About this table

Compound	Synonyms	CAS #	PubMed Ids
Carbon dioxide	Carbon oxide (see all 5)	124-38-9	--
Coproporphyrin III		14643-66-4	--
Coproporphyrinogen III	3,8,13,17-tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoate (see all 9)	2624-63-7	--
Formyl-CoA	Formyl coenzyme A	13131-49-2	--
Heme	(protoporphyrinato)iron (see all 19)	14875-96-8	--
Oxygen	Oxygen (see all 5)	7782-44-7	--
Protoporphyrin IX	3,3'-(3,7,12,17-tetramethyl-8,13-divinyl-21H,23H-porphine-2,18-diyl)-bis-propionate (see all 11)	553-12-8	--
Protoporphyrinogen IX	5,10,15,20,22,24-hexahydro protoporphyrin IX deriv. (see all 8)	7412-77-3	--
Water	Dihydrogen oxide (see all 2)	7732-18-5	--

10/64 Novoseek chemical compound relationships for CPOX gene (see all 64) About this table

Compound	-log (P-Val)	Hits	PubMed IDs for Articles with Shared Sentences (# sentences)
harderoporphyrinogen	91.8	2	17242372 (1)
protoporphyrinogen	86.3	22	12196143 (2), 19339664 (2), 11248690 (1), 8955072 (1) (see all 13)
coprogen	82.9	5	16460507 (2), 16084099 (1), 11374994 (1), 8286403 (1)
prostaglandin	74.2	35	10811855 (2), 16140261 (2), 10651981 (1), 17851120 (1) (see all 24)
porphyrinogen	73.6	2	11909722 (1), 16061377 (1)
uroporphyrinogen-iii	72	4	19289130 (1), 8949908 (1), 8621830 (1)
porphyrin	69.6	14	9298818 (1), 16343843 (1), 19447864 (1), 9674964 (1) (see all 9)
celecoxib	69.1	5	19201774 (2), 20233202 (1), 14762100 (1), 11336575 (1)
porphobilinogen	68.8	7	8024730 (3), 8949908 (1), 10787385 (1)
arachidonic acid	66.1	29	15308583 (2), 8153084 (1), 11230753 (1), 16918423 (1) (see all 23)

Search CenterWatch for drugs/clinical trials and news about CPOX / HEM6

(Secondary structures according to fRNAdb,
GenBank/EMBL/DDBJ Accessions according to
Unigene (Build 235 Homo sapiens; Mar 10 2013) or GenBank,
RefSeq according to Entrez Gene,
DOTS (version 10), and/or AceView, transcript ids from Ensembl with links to UCSC,
exon structure from GeneLoc, alternative splicing isoforms according to ASD and/or ECgene,
RNAi Products from EMD Millipore,
siRNAs from OriGene, QIAGEN, shRNA from OriGene, microRNA from QIAGEN,
Tagged/untagged cDNA clones from OriGene, SwitchGear Genomics, GenScript, DNA2.0, Vector BioLabs, Primers from OriGene, SABiosciences, and/or QIAGEN )
About This Section

REFSEQ mRNAs for CPOX gene:

NM_000097.5

Unigene Cluster for CPOX:

Coproporphyrinogen oxidase

Hs.476982 [show with all ESTs]

Unigene Representative Sequence: NM_000097

5 Ensembl transcripts including schematic representations, and UCSC links where relevant:

ENST00000512905 ENST00000264193(uc003dsx.3) ENST00000510489 ENST00000513674

ENST00000515041(uc011bgz.2)

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		8/13 QIAGEN miScript miRNA Assays for microRNAs that regulate CPOX (see all 13):
		hsa-miR-3163 hsa-miR-361-5p hsa-miR-130a* hsa-miR-3653 hsa-miR-216b hsa-miR-23c hsa-miR-4282 hsa-miR-890

		SwitchGear 3'UTR luciferase reporter plasmid: CPOX 3' UTR sequence

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Additional cDNA sequence:

AK223481.1 AK290140.1 AK299692.1 BC017210.2 BC023551.2 BC023554.2 BX648974.1 D16611.1

Z28409.1

10 DOTS entries:

DT.314896 DT.120889704 DT.100785680 DT.100785681 DT.97842703 DT.120889699 DT.91750697 DT.91750696

DT.92061291 DT.99969577

24/135 AceView cDNA sequences (see all 135):

AL709894 BQ025229 AV653084 AL043660 NM_000097 BU170210 BU624260 W80532

AA700808 N90153 H28785 BF733155 BG107995 BM971248 BF431018 BX494840

BM853610 AV646122 BV204432 CK820489 BG498060 BG944756 N46656 BI711615

GeneLoc Exon Structure

4 Alternative Splicing Database (ASD) splice patterns (SP) for CPOX About this scheme

ExUns:	1a	·	1b	·	1c	·	1d	^	2a	·	2b	·	2c	^	3a	·	3b	^	4a	·	4b	^	5	^	6a	·	6b	^	7
SP1:																									-
SP2:													-		-
SP3:
SP4:							-		-

ECgene alternative splicing isoforms for CPOX

(RNA expression data according to H-InvDB, NONCODE, miRBase, and RNAdb, Expression images according to data from BioGPS, Illumina Human BodyMap, and CGAP SAGE, Sets of similar genes according to GeneDecks, in vivo and in vitro expression data from LifeMap Discovery™, plus additional links to Genevestigator, and/or SOURCE, and/or BioGPS, and/or UniProtKB,
PCR Arrays from SABiosciences, Primers from OriGene, SABiosciences, and/or QIAGEN, In Situ Hybridization Assays from Advanced Cell Diagnostics)
About This Section

CPOX expression in normal human tissues (normalized intensities)
See probesets specificity/sensitivity at GeneAnnot
About this imageBioGPS
CGAP TAG: AATTTAGGCA

About this image
See CPOX Protein Expression from SPIRE MOPED and PaxDB
Genevestigator expression for CPOX

SOURCE GeneReport for Unigene cluster: Hs.476982

SABiosciences Custom PCR Arrays for CPOX

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In Situ
Assay Products:

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(Orthologs according to ^1,2HomoloGene (²older version, for species not in ¹newer version), ³euGenes, ⁴SGD , ⁵MGI Mar 06 2013, with possible further links to Flybase and/or WormBase, and/or ⁶Ensembl pan taxonomic compara , Gene Trees according to Ensembl and TreeFam)
About This Section

This gene was present in the last universal common ancestor (LUCA).

Orthologs for CPOX gene from 9/36 species (see all 36) About this table

Organism	Taxonomic classification	Gene	Description	Human Similarity	Orthology Type	Details
mouse (Mus musculus)	Mammalia	Cpox^{1 , 5}	coproporphyrinogen oxidase^{1, 5}	83.41(n)¹ 81.9(a)¹		16 (34.80 cM)⁵ 12892¹ NM_007757.2¹ NP_031783.2¹ 58670208⁵
chicken (Gallus gallus)	Aves	CPOX¹	coproporphyrinogen oxidase	73.67(n) 76.33(a)		418377 XM_416596.3 XP_416596.1
lizard (Anolis carolinensis)	Reptilia	CPOX⁶	--	86(a)	1 ↔ 1	3(175006081-175012188)
zebrafish (Danio rerio)	Actinopterygii	57049811²	--	79.53(n)		321294 57049811
fruit fly (Drosophila melanogaster)	Insecta	Coprox¹	Coproporphyrinogen oxidase	60.43(n) 63.41(a)		44701 NM_080038.3 NP_524777.1
baker's yeast (Saccharomyces cerevisiae)	Saccharomycetes	HEM13(YDR044W)⁴ HEM13¹	Coproporphyrinogen III oxidase, an oxygen requiring more⁴ Hem13p¹	54.68(n)¹ 54.58(a)¹		4(546642-547628)⁴ 851614^{1, 4} NP_010329.1^{1, 4}
thale cress (Arabidopsis thaliana)	eudicotyledons	LIN2¹	Coproporphyrinogen III oxidase	54.86(n) 54.76(a)		839489 NM_100230.6 NP_171847.4
rice (Oryza sativa)	Liliopsida	Os.17919²	Oryza sativa (japonica cultivar-group) isolate 23485 more	73.97(n)		AK070391.1
E. coli (Escherichia coli)	Gamma proteobacteria	hemF⁶	coproporphyrinogen III oxidase	48(a)	1 ↔ 1	Chromosome(2551247-2552146)

ENSEMBL Gene Tree for CPOX (if available)
TreeFam Gene Tree for CPOX (if available)

(Paralogs according to ¹HomoloGene,
²Ensembl, and ³SIMAP, Pseudogenes according to ⁴Pseudogene.org Build 68)
About This Section

(SNPs/Variants according to the ¹NCBI SNP Database, ²Ensembl, ³PupaSUITE, UniProtKB, and DNA2.0, Linkage Disequilibrium by HapMap, Structural Variations(CNVs/InDels/Inversions) from the Database of Genomic Variants, Mutations from the Human Gene Mutation Database (HGMD) and the Locus Specific Mutation Databases (LSDB), Blood group antigen gene mutations by BGMUT, Resequencing Primers from QIAGEN, Cancer Mutation PCR Arrays and Assays and Copy Number PCR Arrays from SABiosciences)
About This Section

SNP ID	Valid	Clinical significance	Chr 3 pos	Sequence	#	AA Chg	Type	More	#	Allele freq	Pop	Total sample	More
Genomic Data					Transcription Related Data				Allele Frequencies
				--	--	--		--	--
rs121917871^1,2	C	pathogenic	95879269(-)	`GGTCCC/TAGTGC`	2	Q *	stg¹		0	--	--	--	--
rs121917872^1,2	C	pathogenic	95881594(-)	`CATTTC/TTGTTG`	2	S F	mis¹		0	--	--	--	--
rs121917874^1,2	C	pathogenic	95883852(-)	`TTGGTC/GGTGGA`	2	R G	mis¹		0	--	--	--	--
rs121917870^1,2	C	pathogenic	95883900(-)	`CTGTCC/GATTTT`	2	H D	mis¹		0	--	--	--	--
rs121917873^1,2	C	pathogenic	95887056(-)	`CCCATC/TGTGGA`	2	R C	mis¹		0	--	--	--	--
rs121917866^1,2	C	pathogenic	95887065(-)	`GAGAAC/TGGCGG`	2	R W	mis¹		0	--	--	--	--
rs121917868^1,2	C	pathogenic	95891213(-)	`GCACAA/GAGTTT`	2	K E	mis¹		1		NA	4550
rs28931603^1,2	C	pathogenic	95891978(-)	`TTCTAC/TGCCAT`	2	R C	mis¹ ese³		2		NA	4
rs73133922^1,2	C,	--	95670551(+)	`ATGTAC/TGAGAT`	1	--	ut3¹		0	--	--	--	--
rs72924722^1,2	C,	--	95670977(+)	`TTCCAC/TAAAAA`	1	--	ut3¹		4		WA CSA EA	241

HapMap Linkage Disequilibrium report for CPOX (98239976 - 98312567 bp)
Structural Variations (Copy Number Variations, Insertions/Deletions, Inversions)
Database of Genomic Variants (DGV) variations for CPOX: --
Human Gene Mutation Database (HGMD): CPOX

SABiosciences Cancer Mutation PCR Assays

QIAGEN SeqTarget long-range PCR primers in human, mouse, rat for resequencing CPOX

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(in which this Gene is Involved, According to MalaCards, OMIM, UniProtKB, the University of Copenhagen DISEASES database, Novoseek, Genatlas, GeneTests, GAD, HuGE Navigator, and/or TGDB.)
About This Section

CPOX for disorders           About GeneDecksing

OMIM gene information: 612732
OMIM disorders: 121300
UniProtKB/Swiss-Prot: HEM6_HUMAN, P36551

Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic

porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity.

Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically

characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are

generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection,

endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset

attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase

activity

20/69 diseases for CPOX (see all 69): About MalaCards

coproporphyria hereditary coproporphyria kearns-sayre syndrome harderoporphyria

xeroderma pigmentosum, group a porphyria cutanea tarda porphyria congenital erythropoietic porphyria

erythropoietic protoporphyria mitochondrial encephalomyopathy xeroderma pigmentosum acute intermittent porphyria

pre-eclampsia familial adenomatous polyposis encephalomyopathy acute hepatic porphyria

autosomal dominant disease melas syndrome merrf syndrome peptic ulcer

5 diseases from the University of Copenhagen DISEASES database for CPOX:

Hereditary coproporphyria Variegate porphyria Acute intermittent porphyria Erythropoietic protoporphyria

Porphyria cutanea tarda

10/44 Novoseek disease relationships for CPOX gene (see all 44) About this table

Disease	-log (P-Val)	Hits	PubMed IDs for Articles with Shared Sentences (# sentences)
coproporphyria hereditary	96.5	40	7592568 (2), 9843038 (2), 16176984 (2), 8012360 (2) (see all 26)
porphyria hepatic	84.5	6	9454777 (2), 16159891 (1), 8024730 (1), 7757079 (1) (see all 5)
coproporphyria	78.2	1	10899392 (1)
porphyria	74.4	6	16398658 (2), 7592568 (1), 14669009 (1)
porphyria variegate	68.6	2	14669009 (1)
mitochondrial encephalomyopathies	65	3	10637838 (2), 8064325 (1)
porphyria acute intermittent	64.2	3	14669009 (1), 19460837 (1)
oxidase deficiency	57	2	11929047 (1), 8621830 (1)
leigh syndrome	56.8	8	19579665 (2), 7502989 (1), 19772191 (1)
mitochondrial myopathies	52.7	1	10980727 (1)

Human Genome Epidemiology (HuGE) Navigator: CPOX (2 documents)

Export disorders for CPOX gene to outside databases

(in PubMed. Associations of this gene to articles via ¹Entrez Gene, ²UniProtKB/Swiss-Prot, ³HGNC, ⁴GAD, ⁵PharmGKB, ⁶HMDB, ⁷DrugBank, ⁸UniProtKB/TrEMBL, ⁹ Novoseek, and/or ¹⁰fRNAdb)
About This Section

PubMed articles for CPOX gene, integrated from 9 sources (see all 265):
(articles sorted by number of sources associating them with CPOX)

Utopia: connect your pdf to the dynamic
world of online information

A molecular defect in coproporphyrinogen oxidase gene causing harderoporphyria, a variant form of hereditary coproporphyria. (PubMed id 7757079)^{1, 2, 3, 9} Lamoril J....Nordmann Y. (1995)
Characterization and expression of cDNA encoding coproporphyrinogen oxidase from a patient with hereditary coproporphyria. (PubMed id 7849704)^{1, 2, 9} Fujita H.... Sassa S. (1994)
Structural basis of hereditary coproporphyria. (PubMed id 16176984)^{1, 2, 9} Lee D.-S....Raman C.S. (2005)
The long, but not the short, presequence of human coproporphyrinogen oxidase is essential for its import and sorting to mitochondria. (PubMed id 12862310)^{1, 2, 9} Susa S.... Kato T. (2003)
Homozygous hereditary coproporphyria caused by an arginine to tryptophane substitution in coproporphyrinogen oxidase and common intragenic polymorphisms. (PubMed id 8012360)^{1, 2, 9} Martasek P.... Grandchamp B. (1994)
Molecular cloning, sequencing, and functional expression of a cDNA encoding human coproporphyrinogen oxidase. (PubMed id 8159699)^{1, 2, 9} Martasek P.... Grandchamp B. (1994)
Systematic analysis of coproporphyrinogen oxidase gene defects in hereditary coproporphyria and mutation update. (PubMed id 9888388)^{1, 2, 9} Rosipal R.... Deybach J.-C. (1999)
Two novel mutations and coexistence of the 991C>T and the 1339C>T mutation on a single allele in the coproporphyrinogen oxidase gene in Swedish patients with hereditary coproporphyria. (PubMed id 12181641)^{1, 2, 9} Wiman A.... Harper P. (2002)
Molecular cloning, sequencing and expression of cDNA encoding human coproporphyrinogen oxidase. (PubMed id 8286403)^{1, 2, 9} Taketani S.... Tokunaga R. (1994)
Hereditary coproporphyria: exon screening by heteroduplex analysis detects three novel mutations in the coproporphyrinogen oxidase gene. (PubMed id 9298818)^{1, 2, 9} Schreiber W.E.... Jamani A. (1997)

(in PubMed, OMIM, and NCBI Bookshelf)
About This Section

		AND	OR
Aliases
Disorders
Free Text

Query String		PubMed OMIM NCBI Bookshelf
	(Note: In FireFox, select the above section and copy using Ctrl-C)

(According to Entrez Gene, HGNC, AceView, euGenes, Ensembl, miRBase, ECgene, Kegg, and/or H-InvDB)
About This Section

Entrez Gene: 1371	HGNC: 2321	AceView: CPOX	Ensembl:ENSG00000080819	euGenes: HUgn1371
ECgene: CPOX	Kegg: 1371	H-InvDB: CPOX

(According to HUGE)
About This Section

(According to PharmGKB, ATLAS, HORDE, IMGT, LEIDEN, UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL,
Wikipedia and/or GeneReviews via UniProtKB/Swiss-Prot)
About This Section

Name	Description
PharmGKB entry for CPOX	Pharmacogenomics, SNPs, Pathways
GeneReviews	http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/CPOX

Intellectual Property for CPOX gene
(Patent information from GeneIP, Licensable technologies from WIS Yeda, Salk, Tufts, IP news from LifeMap Sciences, Inc.)

About This Section

Patent Information for CPOX gene:
Search GeneIP for patents involving CPOX

GeneCards and IP:
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MERRF
Gastric Cancer
Porphyria
BREAST CARCINOMA
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Anemia Hemolytic
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PORPHYRIA, CONGENITAL ERYTHROPOIETIC
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Protoporphyria Erythropoietic
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CPOX Gene

coproporphyrinogen oxidase

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