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CPOX (coproporphyrinogen oxidase)

Gene

1371

Coproporphyrinogen oxidase

CPOX

COX, CPO, CPX, HARPO, HCP

3q11.2

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 I

Show/Hide all(19)

SNP ID	Visualize variation	Clinical significance	Consequence
rs28931603	G>A	Pathogenic	Non coding transcript variant, intron variant, missense variant, coding sequence variant
rs121917866	G>A	Pathogenic	Non coding transcript variant, missense variant, coding sequence variant
rs121917868	T>C	Pathogenic, likely-pathogenic	Non coding transcript variant, missense variant, intron variant, coding sequence variant
rs121917870	G>A,C	Pathogenic	Non coding transcript variant, missense variant, coding sequence variant
rs121917871	G>A,C	Pathogenic	Non coding transcript variant, stop gained, missense variant, coding sequence variant
rs121917872	G>A	Pathogenic	Non coding transcript variant, missense variant, coding sequence variant
rs121917873	G>A	Pathogenic	Non coding transcript variant, missense variant, coding sequence variant
rs121917874	C>G	Pathogenic	Non coding transcript variant, missense variant, coding sequence variant
rs370245685	T>C	Pathogenic	Intron variant
rs576756431	A>T	Pathogenic	Stop gained, coding sequence variant, non coding transcript variant
rs587777271	T>C	Pathogenic	Missense variant, coding sequence variant, non coding transcript variant
rs781627991	C>G,T	Conflicting-interpretations-of-pathogenicity	Missense variant, non coding transcript variant, coding sequence variant
rs786205053	->CGCTG	Pathogenic	Frameshift variant, non coding transcript variant, coding sequence variant
rs786205054	TGCCAGAGCCTGGCACACCTG>-	Pathogenic	Inframe deletion, non coding transcript variant, coding sequence variant
rs1553696121	->G	Likely-pathogenic	Frameshift variant, non coding transcript variant, coding sequence variant
rs1559677768	->T	Pathogenic	Non coding transcript variant, frameshift variant, coding sequence variant
rs1576304776	A>C	Likely-pathogenic	Splice donor variant
rs1576306536	G>A	Pathogenic	Non coding transcript variant, coding sequence variant, stop gained
rs1576306935	C>-	Pathogenic	Frameshift variant, non coding transcript variant, coding sequence variant

Show/Hide all(339)

miRTarBase ID	miRNA	Experiments	Reference
MIRT001374	hsa-miR-1-3p	pSILAC	18668040
MIRT019712	hsa-miR-375	Microarray	20215506
MIRT020103	hsa-miR-361-5p	Sequencing	20371350
MIRT022424	hsa-miR-124-3p	Microarray	18668037
MIRT001374	hsa-miR-1-3p	Proteomics;Other	18668040
MIRT027462	hsa-miR-98-5p	Microarray	19088304
MIRT028461	hsa-miR-30a-5p	Proteomics	18668040
MIRT047853	hsa-miR-30c-5p	CLASH	23622248
MIRT046519	hsa-miR-15b-5p	CLASH	23622248
MIRT036635	hsa-miR-937-3p	CLASH	23622248
MIRT450431	hsa-miR-5582-3p	PAR-CLIP	22100165
MIRT450430	hsa-miR-1323	PAR-CLIP	22100165
MIRT450429	hsa-miR-548o-3p	PAR-CLIP	22100165
MIRT450428	hsa-miR-643	PAR-CLIP	22100165
MIRT450427	hsa-miR-526b-5p	PAR-CLIP	22100165
MIRT450426	hsa-miR-6869-5p	PAR-CLIP	22100165
MIRT450425	hsa-miR-578	PAR-CLIP	22100165
MIRT440887	hsa-miR-106b-5p	HITS-CLIP	22473208
MIRT440886	hsa-miR-20a-5p	HITS-CLIP	22473208
MIRT440884	hsa-miR-218-5p	HITS-CLIP	23212916
MIRT440887	hsa-miR-106b-5p	HITS-CLIP	22473208
MIRT727949	hsa-miR-17-5p	HITS-CLIP	22473208
MIRT727948	hsa-miR-20b-5p	HITS-CLIP	22473208
MIRT727947	hsa-miR-93-5p	HITS-CLIP	22473208
MIRT727946	hsa-miR-519d-3p	HITS-CLIP	22473208
MIRT727945	hsa-miR-181a-5p	HITS-CLIP	22473208
MIRT727943	hsa-miR-181b-5p	HITS-CLIP	22473208
MIRT727944	hsa-miR-181c-5p	HITS-CLIP	22473208
MIRT727942	hsa-miR-181d-5p	HITS-CLIP	22473208
MIRT440886	hsa-miR-20a-5p	HITS-CLIP	22473208
MIRT440884	hsa-miR-218-5p	HITS-CLIP	23212916
MIRT450431	hsa-miR-5582-3p	PAR-CLIP	22100165
MIRT450430	hsa-miR-1323	PAR-CLIP	22100165
MIRT450429	hsa-miR-548o-3p	PAR-CLIP	22100165
MIRT450428	hsa-miR-643	PAR-CLIP	22100165
MIRT450427	hsa-miR-526b-5p	PAR-CLIP	22100165
MIRT450426	hsa-miR-6869-5p	PAR-CLIP	22100165
MIRT450425	hsa-miR-578	PAR-CLIP	22100165
MIRT906788	hsa-miR-106a	CLIP-seq
MIRT906789	hsa-miR-106b	CLIP-seq
MIRT906790	hsa-miR-1200	CLIP-seq
MIRT906791	hsa-miR-1305	CLIP-seq
MIRT906792	hsa-miR-17	CLIP-seq
MIRT906793	hsa-miR-181a	CLIP-seq
MIRT906794	hsa-miR-181b	CLIP-seq
MIRT906795	hsa-miR-181c	CLIP-seq
MIRT906796	hsa-miR-181d	CLIP-seq
MIRT906797	hsa-miR-200b	CLIP-seq
MIRT906798	hsa-miR-200c	CLIP-seq
MIRT906799	hsa-miR-204	CLIP-seq
MIRT906800	hsa-miR-20a	CLIP-seq
MIRT906801	hsa-miR-20b	CLIP-seq
MIRT906802	hsa-miR-211	CLIP-seq
MIRT906803	hsa-miR-2113	CLIP-seq
MIRT906804	hsa-miR-23a	CLIP-seq
MIRT906805	hsa-miR-23b	CLIP-seq
MIRT906806	hsa-miR-23c	CLIP-seq
MIRT906807	hsa-miR-2467-3p	CLIP-seq
MIRT906808	hsa-miR-3074-5p	CLIP-seq
MIRT906809	hsa-miR-30a	CLIP-seq
MIRT906810	hsa-miR-30b	CLIP-seq
MIRT906811	hsa-miR-30c	CLIP-seq
MIRT906812	hsa-miR-30d	CLIP-seq
MIRT906813	hsa-miR-30e	CLIP-seq
MIRT906814	hsa-miR-3117-5p	CLIP-seq
MIRT906815	hsa-miR-3123	CLIP-seq
MIRT906816	hsa-miR-3127-5p	CLIP-seq
MIRT906817	hsa-miR-3163	CLIP-seq
MIRT906818	hsa-miR-323-3p	CLIP-seq
MIRT906819	hsa-miR-3646	CLIP-seq
MIRT906820	hsa-miR-3653	CLIP-seq
MIRT906821	hsa-miR-3657	CLIP-seq
MIRT906822	hsa-miR-3658	CLIP-seq
MIRT906823	hsa-miR-3662	CLIP-seq
MIRT906824	hsa-miR-3678-3p	CLIP-seq
MIRT906825	hsa-miR-3685	CLIP-seq
MIRT906826	hsa-miR-3688-5p	CLIP-seq
MIRT906827	hsa-miR-369-3p	CLIP-seq
MIRT906828	hsa-miR-374a	CLIP-seq
MIRT906829	hsa-miR-374b	CLIP-seq
MIRT906830	hsa-miR-384	CLIP-seq
MIRT906831	hsa-miR-3913-3p	CLIP-seq
MIRT906832	hsa-miR-3919	CLIP-seq
MIRT906833	hsa-miR-3938	CLIP-seq
MIRT906834	hsa-miR-3978	CLIP-seq
MIRT906835	hsa-miR-4261	CLIP-seq
MIRT906836	hsa-miR-4262	CLIP-seq
MIRT906837	hsa-miR-4270	CLIP-seq
MIRT906838	hsa-miR-429	CLIP-seq
MIRT906839	hsa-miR-433	CLIP-seq
MIRT906840	hsa-miR-4441	CLIP-seq
MIRT906841	hsa-miR-4474-3p	CLIP-seq
MIRT906842	hsa-miR-4669	CLIP-seq
MIRT906843	hsa-miR-4704-5p	CLIP-seq
MIRT906844	hsa-miR-4712-3p	CLIP-seq
MIRT906845	hsa-miR-4719	CLIP-seq
MIRT906846	hsa-miR-4733-5p	CLIP-seq
MIRT906847	hsa-miR-4755-5p	CLIP-seq
MIRT906848	hsa-miR-4760-3p	CLIP-seq
MIRT906849	hsa-miR-4774-3p	CLIP-seq
MIRT906850	hsa-miR-485-3p	CLIP-seq
MIRT906851	hsa-miR-487a	CLIP-seq
MIRT906852	hsa-miR-489	CLIP-seq
MIRT906853	hsa-miR-519a	CLIP-seq
MIRT906854	hsa-miR-519b-3p	CLIP-seq
MIRT906855	hsa-miR-519c-3p	CLIP-seq
MIRT906856	hsa-miR-519d	CLIP-seq
MIRT906857	hsa-miR-548t	CLIP-seq
MIRT906858	hsa-miR-570	CLIP-seq
MIRT906859	hsa-miR-623	CLIP-seq
MIRT906860	hsa-miR-676	CLIP-seq
MIRT906861	hsa-miR-890	CLIP-seq
MIRT906862	hsa-miR-93	CLIP-seq
MIRT906788	hsa-miR-106a	CLIP-seq
MIRT906789	hsa-miR-106b	CLIP-seq
MIRT906790	hsa-miR-1200	CLIP-seq
MIRT906792	hsa-miR-17	CLIP-seq
MIRT906793	hsa-miR-181a	CLIP-seq
MIRT906794	hsa-miR-181b	CLIP-seq
MIRT906795	hsa-miR-181c	CLIP-seq
MIRT906796	hsa-miR-181d	CLIP-seq
MIRT1968725	hsa-miR-190	CLIP-seq
MIRT1968726	hsa-miR-190b	CLIP-seq
MIRT906800	hsa-miR-20a	CLIP-seq
MIRT906801	hsa-miR-20b	CLIP-seq
MIRT906804	hsa-miR-23a	CLIP-seq
MIRT906805	hsa-miR-23b	CLIP-seq
MIRT906806	hsa-miR-23c	CLIP-seq
MIRT906807	hsa-miR-2467-3p	CLIP-seq
MIRT906808	hsa-miR-3074-5p	CLIP-seq
MIRT906814	hsa-miR-3117-5p	CLIP-seq
MIRT1968727	hsa-miR-3121-3p	CLIP-seq
MIRT906815	hsa-miR-3123	CLIP-seq
MIRT906817	hsa-miR-3163	CLIP-seq
MIRT906819	hsa-miR-3646	CLIP-seq
MIRT1968728	hsa-miR-3660	CLIP-seq
MIRT906824	hsa-miR-3678-3p	CLIP-seq
MIRT1968729	hsa-miR-3691-3p	CLIP-seq
MIRT1968730	hsa-miR-3692	CLIP-seq
MIRT906832	hsa-miR-3919	CLIP-seq
MIRT906836	hsa-miR-4262	CLIP-seq
MIRT1968731	hsa-miR-4272	CLIP-seq
MIRT1968732	hsa-miR-4522	CLIP-seq
MIRT1968733	hsa-miR-4526	CLIP-seq
MIRT906843	hsa-miR-4704-5p	CLIP-seq
MIRT906844	hsa-miR-4712-3p	CLIP-seq
MIRT1968734	hsa-miR-4714-5p	CLIP-seq
MIRT906846	hsa-miR-4733-5p	CLIP-seq
MIRT1968735	hsa-miR-4756-3p	CLIP-seq
MIRT1968736	hsa-miR-4766-5p	CLIP-seq
MIRT906853	hsa-miR-519a	CLIP-seq
MIRT906854	hsa-miR-519b-3p	CLIP-seq
MIRT906855	hsa-miR-519c-3p	CLIP-seq
MIRT906856	hsa-miR-519d	CLIP-seq
MIRT906857	hsa-miR-548t	CLIP-seq
MIRT1968737	hsa-miR-654-3p	CLIP-seq
MIRT906862	hsa-miR-93	CLIP-seq
MIRT1968738	hsa-miR-95	CLIP-seq
MIRT2204859	hsa-let-7a	CLIP-seq
MIRT2204860	hsa-let-7b	CLIP-seq
MIRT2204861	hsa-let-7c	CLIP-seq
MIRT2204862	hsa-let-7d	CLIP-seq
MIRT2204863	hsa-let-7e	CLIP-seq
MIRT2204864	hsa-let-7f	CLIP-seq
MIRT2204865	hsa-let-7g	CLIP-seq
MIRT2204866	hsa-let-7i	CLIP-seq
MIRT906788	hsa-miR-106a	CLIP-seq
MIRT906789	hsa-miR-106b	CLIP-seq
MIRT2204867	hsa-miR-1229	CLIP-seq
MIRT2204868	hsa-miR-1253	CLIP-seq
MIRT2204869	hsa-miR-1297	CLIP-seq
MIRT2204870	hsa-miR-1303	CLIP-seq
MIRT906792	hsa-miR-17	CLIP-seq
MIRT906793	hsa-miR-181a	CLIP-seq
MIRT906794	hsa-miR-181b	CLIP-seq
MIRT906795	hsa-miR-181c	CLIP-seq
MIRT906796	hsa-miR-181d	CLIP-seq
MIRT1968725	hsa-miR-190	CLIP-seq
MIRT1968726	hsa-miR-190b	CLIP-seq
MIRT2204871	hsa-miR-1913	CLIP-seq
MIRT906800	hsa-miR-20a	CLIP-seq
MIRT906801	hsa-miR-20b	CLIP-seq
MIRT2204872	hsa-miR-2277-3p	CLIP-seq
MIRT906804	hsa-miR-23a	CLIP-seq
MIRT906805	hsa-miR-23b	CLIP-seq
MIRT906806	hsa-miR-23c	CLIP-seq
MIRT906807	hsa-miR-2467-3p	CLIP-seq
MIRT2204873	hsa-miR-26a	CLIP-seq
MIRT2204874	hsa-miR-26b	CLIP-seq
MIRT906809	hsa-miR-30a	CLIP-seq
MIRT906810	hsa-miR-30b	CLIP-seq
MIRT906811	hsa-miR-30c	CLIP-seq
MIRT906812	hsa-miR-30d	CLIP-seq
MIRT906813	hsa-miR-30e	CLIP-seq
MIRT906814	hsa-miR-3117-5p	CLIP-seq
MIRT906815	hsa-miR-3123	CLIP-seq
MIRT906816	hsa-miR-3127-5p	CLIP-seq
MIRT906817	hsa-miR-3163	CLIP-seq
MIRT2204875	hsa-miR-324-3p	CLIP-seq
MIRT906819	hsa-miR-3646	CLIP-seq
MIRT1968728	hsa-miR-3660	CLIP-seq
MIRT906823	hsa-miR-3662	CLIP-seq
MIRT906824	hsa-miR-3678-3p	CLIP-seq
MIRT1968730	hsa-miR-3692	CLIP-seq
MIRT906832	hsa-miR-3919	CLIP-seq
MIRT2204876	hsa-miR-425	CLIP-seq
MIRT906836	hsa-miR-4262	CLIP-seq
MIRT906837	hsa-miR-4270	CLIP-seq
MIRT2204877	hsa-miR-4420	CLIP-seq
MIRT2204878	hsa-miR-4434	CLIP-seq
MIRT906840	hsa-miR-4441	CLIP-seq
MIRT2204879	hsa-miR-4458	CLIP-seq
MIRT2204880	hsa-miR-4465	CLIP-seq
MIRT2204881	hsa-miR-4500	CLIP-seq
MIRT2204882	hsa-miR-4516	CLIP-seq
MIRT1968732	hsa-miR-4522	CLIP-seq
MIRT1968733	hsa-miR-4526	CLIP-seq
MIRT2204883	hsa-miR-4531	CLIP-seq
MIRT906843	hsa-miR-4704-5p	CLIP-seq
MIRT1968734	hsa-miR-4714-5p	CLIP-seq
MIRT2204884	hsa-miR-4727-5p	CLIP-seq
MIRT906846	hsa-miR-4733-5p	CLIP-seq
MIRT2204885	hsa-miR-4769-3p	CLIP-seq
MIRT2204886	hsa-miR-495	CLIP-seq
MIRT906853	hsa-miR-519a	CLIP-seq
MIRT906854	hsa-miR-519b-3p	CLIP-seq
MIRT906855	hsa-miR-519c-3p	CLIP-seq
MIRT906856	hsa-miR-519d	CLIP-seq
MIRT2204887	hsa-miR-520d-5p	CLIP-seq
MIRT2204888	hsa-miR-524-5p	CLIP-seq
MIRT906857	hsa-miR-548t	CLIP-seq
MIRT2204889	hsa-miR-668	CLIP-seq
MIRT906860	hsa-miR-676	CLIP-seq
MIRT2204890	hsa-miR-759	CLIP-seq
MIRT906862	hsa-miR-93	CLIP-seq
MIRT1968738	hsa-miR-95	CLIP-seq
MIRT2204891	hsa-miR-98	CLIP-seq
MIRT2386424	hsa-miR-3617	CLIP-seq
MIRT1968728	hsa-miR-3660	CLIP-seq
MIRT1968733	hsa-miR-4526	CLIP-seq
MIRT906845	hsa-miR-4719	CLIP-seq
MIRT2386425	hsa-miR-641	CLIP-seq
MIRT906788	hsa-miR-106a	CLIP-seq
MIRT906789	hsa-miR-106b	CLIP-seq
MIRT906792	hsa-miR-17	CLIP-seq
MIRT906800	hsa-miR-20a	CLIP-seq
MIRT906801	hsa-miR-20b	CLIP-seq
MIRT906807	hsa-miR-2467-3p	CLIP-seq
MIRT906815	hsa-miR-3123	CLIP-seq
MIRT906824	hsa-miR-3678-3p	CLIP-seq
MIRT1968730	hsa-miR-3692	CLIP-seq
MIRT906832	hsa-miR-3919	CLIP-seq
MIRT906846	hsa-miR-4733-5p	CLIP-seq
MIRT2204886	hsa-miR-495	CLIP-seq
MIRT906853	hsa-miR-519a	CLIP-seq
MIRT906854	hsa-miR-519b-3p	CLIP-seq
MIRT906855	hsa-miR-519c-3p	CLIP-seq
MIRT906856	hsa-miR-519d	CLIP-seq
MIRT906857	hsa-miR-548t	CLIP-seq
MIRT906862	hsa-miR-93	CLIP-seq
MIRT906788	hsa-miR-106a	CLIP-seq
MIRT906789	hsa-miR-106b	CLIP-seq
MIRT2508221	hsa-miR-128	CLIP-seq
MIRT2204869	hsa-miR-1297	CLIP-seq
MIRT906792	hsa-miR-17	CLIP-seq
MIRT906800	hsa-miR-20a	CLIP-seq
MIRT906801	hsa-miR-20b	CLIP-seq
MIRT2204872	hsa-miR-2277-3p	CLIP-seq
MIRT2204873	hsa-miR-26a	CLIP-seq
MIRT2204874	hsa-miR-26b	CLIP-seq
MIRT2508222	hsa-miR-3064-3p	CLIP-seq
MIRT2508223	hsa-miR-3150a-3p	CLIP-seq
MIRT2508224	hsa-miR-3175	CLIP-seq
MIRT2508225	hsa-miR-3622b-5p	CLIP-seq
MIRT906826	hsa-miR-3688-5p	CLIP-seq
MIRT2508226	hsa-miR-371-3p	CLIP-seq
MIRT2508227	hsa-miR-3973	CLIP-seq
MIRT2508228	hsa-miR-4282	CLIP-seq
MIRT2508229	hsa-miR-4290	CLIP-seq
MIRT2204880	hsa-miR-4465	CLIP-seq
MIRT2508230	hsa-miR-4470	CLIP-seq
MIRT2508231	hsa-miR-4724-3p	CLIP-seq
MIRT906856	hsa-miR-519d	CLIP-seq
MIRT2508232	hsa-miR-520g	CLIP-seq
MIRT2508233	hsa-miR-520h	CLIP-seq
MIRT2508234	hsa-miR-548a-5p	CLIP-seq
MIRT2508235	hsa-miR-548ab	CLIP-seq
MIRT2508236	hsa-miR-548ak	CLIP-seq
MIRT2508237	hsa-miR-548b-5p	CLIP-seq
MIRT2508238	hsa-miR-548c-5p	CLIP-seq
MIRT2508239	hsa-miR-548d-5p	CLIP-seq
MIRT2508240	hsa-miR-548h	CLIP-seq
MIRT2508241	hsa-miR-548i	CLIP-seq
MIRT2508242	hsa-miR-548j	CLIP-seq
MIRT2508243	hsa-miR-548w	CLIP-seq
MIRT2508244	hsa-miR-548y	CLIP-seq
MIRT2508245	hsa-miR-559	CLIP-seq
MIRT906862	hsa-miR-93	CLIP-seq
MIRT2508246	hsa-miR-939	CLIP-seq
MIRT2204859	hsa-let-7a	CLIP-seq
MIRT2204860	hsa-let-7b	CLIP-seq
MIRT2204861	hsa-let-7c	CLIP-seq
MIRT2204862	hsa-let-7d	CLIP-seq
MIRT2204863	hsa-let-7e	CLIP-seq
MIRT2204864	hsa-let-7f	CLIP-seq
MIRT2204865	hsa-let-7g	CLIP-seq
MIRT2204866	hsa-let-7i	CLIP-seq
MIRT906788	hsa-miR-106a	CLIP-seq
MIRT906789	hsa-miR-106b	CLIP-seq
MIRT2204868	hsa-miR-1253	CLIP-seq
MIRT2508221	hsa-miR-128	CLIP-seq
MIRT906792	hsa-miR-17	CLIP-seq
MIRT2204871	hsa-miR-1913	CLIP-seq
MIRT906800	hsa-miR-20a	CLIP-seq
MIRT906801	hsa-miR-20b	CLIP-seq
MIRT2204872	hsa-miR-2277-3p	CLIP-seq
MIRT906807	hsa-miR-2467-3p	CLIP-seq
MIRT2508222	hsa-miR-3064-3p	CLIP-seq
MIRT2508223	hsa-miR-3150a-3p	CLIP-seq
MIRT2508224	hsa-miR-3175	CLIP-seq
MIRT2204875	hsa-miR-324-3p	CLIP-seq
MIRT2508225	hsa-miR-3622b-5p	CLIP-seq
MIRT906824	hsa-miR-3678-3p	CLIP-seq
MIRT906826	hsa-miR-3688-5p	CLIP-seq
MIRT2508226	hsa-miR-371-3p	CLIP-seq
MIRT2508229	hsa-miR-4290	CLIP-seq
MIRT2204878	hsa-miR-4434	CLIP-seq
MIRT2204879	hsa-miR-4458	CLIP-seq
MIRT2508230	hsa-miR-4470	CLIP-seq
MIRT2204881	hsa-miR-4500	CLIP-seq
MIRT2204882	hsa-miR-4516	CLIP-seq
MIRT2204883	hsa-miR-4531	CLIP-seq
MIRT906856	hsa-miR-519d	CLIP-seq
MIRT2508232	hsa-miR-520g	CLIP-seq
MIRT2508233	hsa-miR-520h	CLIP-seq
MIRT2204890	hsa-miR-759	CLIP-seq
MIRT906862	hsa-miR-93	CLIP-seq
MIRT2508246	hsa-miR-939	CLIP-seq
MIRT2204891	hsa-miR-98	CLIP-seq

Show/Hide all(10)

GO ID	Ontology	Definition	Evidence	Reference
GO:0004109	Function	Coproporphyrinogen oxidase activity	IBA	21873635
GO:0005737	Component	Cytoplasm	IBA	21873635
GO:0005739	Component	Mitochondrion	IDA
GO:0005758	Component	Mitochondrial intermembrane space	IBA	21873635
GO:0005758	Component	Mitochondrial intermembrane space	TAS
GO:0005829	Component	Cytosol	IDA
GO:0006782	Process	Protoporphyrinogen IX biosynthetic process	IBA	21873635
GO:0006782	Process	Protoporphyrinogen IX biosynthetic process	IEA
GO:0006783	Process	Heme biosynthetic process	TAS
GO:0042803	Function	Protein homodimerization activity	IDA	16176984

MIM	HGNC	e!Ensembl
612732	2321	ENSG00000080819

Protein

UniProt ID

P36551

Protein name

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial (COX) (Coprogen oxidase) (Coproporphyrinogenase) (EC 1.3.3.3)

Protein function

Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX and participates to the sixth step in the heme biosynthetic pathway. {ECO:0000269|

PDB

2AEX

Family and domains

Pfam

Accession	ID	Position in sequence	Description	Type
PF01218	Coprogen_oxidas	151 → 453	Coproporphyrinogen III oxidase	Family

Sequence

MALQLGRLSSGPCWLVARGGCGGPRAWSQCGGGGLRAWSQRSAAGRVCRPPGPAGTEQSR
GLGHGSTSRGGPWVGTGLAAALAGLVGLATAAFGHVQRAEMLPKTSGTRATSLGRPEEEE
DELAHRCSSFMAPPVTDLGELRRRPGDMKTKMELLILETQAQVCQALAQVDGGANFSVDR
WERKEGGGGISCVLQDGCVFEKAGVSISVVHGNLSEEAAKQMRSRGKVLKTKDGKLPFCA
MGVSSVIHPKNPHAPTIHFNYRYFEVEEADGNKQWWFGGGCDLTPTYLNQEDAVHFHRTL
KEACDQHGPDLYPKFKKWCDDYFFIAHRGERRGIGGIFFDDLDSPSKEEVFRFVQSCARA
VVPSYIPLVKKHCDDSFTPQEKLWQQLRRGRYVEFNLLYDRGTKFGLFTPGSRIESILMS
LPLTARWEYMHSPSENSKEAEILEVLRHPRDWVR

Sequence length

454

Interactions

View interactions

	KEGG		Reactome
	Porphyrin metabolism Metabolic pathways Biosynthesis of cofactors		Heme biosynthesis

Associated diseases

Show/Hide Causal Diseases (6)

Causal
Disease name	Disease term	dbSNP ID	References
Anemia	Anemia, Hemolytic, Congenital	rs118204044, rs118204045, rs118204046, rs121918330, rs869320719, rs869312029, rs121918332, rs869320724, rs767094129, rs786205058, rs786205059, rs137853119, rs137853120, rs137853121, rs1384933966, rs137853122, rs137853123, rs786205060, rs267607121, rs121908584, rs80338697, rs80338699, rs120074166, rs120074167, rs1050828, rs74575103, rs137852314, rs5030868, rs137852316, rs137852317, rs137852318, rs137852319, rs137852320, rs137852321, rs137852322, rs137852323, rs137852324, rs72554665, rs387906468, rs5030872, rs137852326, rs137852333, rs137852327, rs137852328, rs137852329, rs137852330, rs137852331, rs137852332, rs137852334, rs137852335, rs137852336, rs137852339, rs76645461, rs137852340, rs137852341, rs78478128, rs137852343, rs137852344, rs137852345, rs587776730, rs137852346, rs137852347, rs137852349, rs2070404412, rs2070350038, rs2070350009, rs137852303, rs137852304, rs33946267, rs34378160, rs33933298, rs11549407, rs35724775, rs34598529, rs41469945, rs267607201, rs80338694, rs80338696, rs387907018, rs398123546, rs78365220, rs587777100, rs587777101, rs483352840, rs869312752, rs765487627, rs1557229599, rs1557230040, rs1555524842, rs782090947, rs1358275550, rs1557229736, rs1557230573, rs1556323334, rs1233124208, rs1293528130, rs146864395, rs1595503440, rs1603411214, rs137852325, rs1575247302, rs1603411177, rs1336651679, rs782322505
Coproporphyria	Coproporphyria	rs2107113853, rs786205053, rs786205054, rs121917870, rs121917871, rs121917872, rs121917873, rs1559677768, rs121917874
Harderoporphyria	Harderoporphyria	rs121917868, rs587777271
Hereditary coproporphyria	Hereditary Coproporphyria	rs1576306536, rs1374394802	9048920, 11831056, 8990017, 27604308, 9298818, 9888388, 16398658, 8012360, 15896662, 7849704, 7757079, 12181641
Hypertension	Hypertensive disease	rs13306026, rs13333226
Porphyria cutanea tarda	Porphyria Cutanea Tarda	rs121918057, rs145195562, rs121918062, rs121918063, rs121918064, rs121918065, rs397514764, rs397514765, rs143823335, rs1569967143	11831056

Show/Hide Unknown Diseases (6)

Unknown
Disease name	Disease term	dbSNP ID	References
Anxiety disorder	Anxiety
Hallucinations	Hallucinations
Hypertrichosis	Hypertrichosis
Mental depression	Depressive disorder	rs587778876, rs587778877
Nervous system diseases	Peripheral Nervous System Diseases
Paranoia	Paranoia

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