| 1321106 | COX10 | cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 | Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits e ncoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion. [provided by RefSeq, Jul 2008] | 17 | 14069504 | 14208677 | Human | 217 | symbol , old_gene_name , COSMIC , name , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1321107 | Cox10 | heme A:farnesyltransferase cytochrome c oxidase assembly factor 10 | Enables protoheme IX farnesyltransferase activity. Involved in heme O biosynthetic process. Acts upstream of or within several processes, including copper ion homeostasis; heme A biosynthetic process; and mitochondrial fission. Located in mitochondrion. Is active in mitochondrial matrix. Is expresse d in alimentary system; nervous system; respiratory system; and submandibular gland primordium. Used to study Parkinson's disease; cytochrome-c oxidase deficiency disease; and mitochondrial myopathy. Human ortholog(s) of this gene implicated in mitochondrial complex IV deficiency nuclear type 3. Orthologous to human COX10 (cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10). [provided by Alliance of Genome Resources, Apr 2025] | 11 | 63853453 | 63970298 | Mouse | 162 | symbol , old_gene_name , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 1594623 | Cox10 | cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (ortholog); protoheme IX farnesyltransferase activity (ortholog); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent por phyria pathway; erythropoietic porphyria pathway; hereditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (ortholog); mitochondrial matrix (ortholog); mitochondrion (ortholog); INTERACTS WITH 2,3,7,8-tetrachlorodibenzodioxine; amphetamine; atrazine | 10 | 49130209 | 49242009 | Rat | 108 | symbol , old_gene_name , PhenoGen , name | gene, protein-coding, VALIDATED [RefSeq] |
| 12361943 | COX10 | cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (inferred); prenyltransferase activity (inferred); protoheme IX farnesyltransferase activity (inferred); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent porphyria pathway; erythropoietic porphyria pathway; hereditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (inferred); membrane (inferred); mitochondrial matrix (inferred) | 5 | 37853495 | 37982228 | Dog | 44 | symbol , old_gene_name , name | gene, protein-coding, MODEL [RefSeq] |
| 14371639 | COX10 | | | | | | Yeast | | symbol | gene, null |
| 13889863 | COX10 | cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (inferred); prenyltransferase activity (inferred); protoheme IX farnesyltransferase activity (inferred); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent porphyria pathway; erythropoietic porphyria pathway; hereditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (inferred); membrane (inferred); mitochondrial matrix (inferred); INTERACTS WITH deoxynivalenol | | | | Pig | 46 | symbol , old_gene_name , name | gene, protein-coding, VALIDATED [RefSeq] |
| 287814292 | cox10 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 18622559 | COX10 | cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10 | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (inferred); prenyltransferase activity (inferred); protoheme IX farnesyltransferase activity (inferred); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent porphyria pathway; erythropoietic porphyria pathway; hereditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (inferred); membrane (inferred); mitochondrial matrix (inferred) | | | | Green Monkey | 46 | symbol , old_gene_name , name | gene, protein-coding, MODEL [RefSeq] |
| 284133431 | cox10.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 284371657 | cox10.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 6481261 | COX10-DT | COX10 divergent transcript | ASSOCIATED WITH cytochrome-c oxidase deficiency disease; INTERACTS WITH 2-hydroxypropanoic acid; rac-lactic acid; sodium dodecyl sulfate | 17 | 14029292 | 14069458 | Human | 4 | symbol , old_gene_name , COSMIC , name , Human Proteome Map , old_gene_symbol | gene, ncrna, VALIDATED [RefSeq] |
| 18932752 | LOC101726271 | protoheme IX farnesyltransferase, mitochondrial | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (ortholog); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent porphyria pathway; erythropoietic porphyria pathway; here ditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (ortholog) | | | | Naked Mole-Rat | 45 | old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 16552626 | AC005838.1 | pseudogene fragment similar to COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast) (COX10) | | | | | Human | | name | gene, unprocessed_pseudogene |
| 16554066 | AL355355.1 | pseudogene similar to part of COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast) (COX10) | | | | | Human | | name | gene, processed_pseudogene |
| 11936438 | LOC100980340 | protoheme IX farnesyltransferase, mitochondrial | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (inferred); prenyltransferase activity (inferred); protoheme IX farnesyltransferase activity (inferred); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent porphyria pathway; erythropoietic porphyria pathway; hereditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (inferred); membrane (inferred); mitochondrial matrix (inferred) | 17 | 37443319 | 37582436 | Bonobo | 46 | ensembl_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 8950268 | LOC102022268 | protoheme IX farnesyltransferase, mitochondrial | ENCODES a protein that exhibits geranylgeranyl diphosphate synthase activity (ortholog); protoheme IX farnesyltransferase activity (ortholog); INVOLVED IN aerobic respiration (ortholog); copper ion homeostasis (ortholog); cytochrome complex assembly (ortholog); PARTICIPATES IN acute intermittent por phyria pathway; erythropoietic porphyria pathway; hereditary coproporphyria pathway; ASSOCIATED WITH cytochrome-c oxidase deficiency disease (ortholog); genetic disease (ortholog); Leigh disease (ortholog); FOUND IN cytochrome complex (ortholog); mitochondrial matrix (ortholog); mitochondrion (ortholog) | NW_004955467 | 3294200 | 3415778 | Chinchilla | 32 | ensembl_gene_symbol | gene, protein-coding, MODEL [RefSeq] |