| 2381 | Coq7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits 3-demethoxyubiquinone 3-hydroxylase (NADH) activity (ortholog); chromatin binding (ortholog); oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen (ortholog ); INVOLVED IN response to xenobiotic stimulus; ubiquinone biosynthetic process; cellular response to oxidative stress (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH Left Ventricular Hypertrophy; autosomal recessive distal hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrial inner membrane (ortholog); mitochondrion (ortholog); INTERACTS WITH 17alpha-ethynylestradiol; 2,3,7,8-tetrachlorodibenzodioxine; 6-propyl-2-thiouracil | 1 | 182270570 | 182285959 | Rat | 131 | symbol , old_gene_name , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 10379 | Coq7 | demethyl-Q 7 | Enables oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen. Involved in ubiquinone biosynthetic process. Acts upstream of or within several processes, including chordate embryonic develo pment; determination of adult lifespan; and mitochondrial ATP synthesis coupled electron transport. Located in mitochondrion. Is extrinsic component of mitochondrial inner membrane. Is expressed in several structures, including alimentary system; ear; heart; respiratory system; and skeleton. Human ortholog(s) of this gene implicated in autosomal recessive distal hereditary motor neuronopathy 9 and primary coenzyme Q10 deficiency 8. Orthologous to human COQ7 (coenzyme Q7, hydroxylase). [provided by Alliance of Genome Resources, Jul 2025] | 7 | 118108881 | 118132529 | Mouse | 157 | symbol , old_gene_name , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 737189 | COQ7 | coenzyme Q7, hydroxylase | The protein encoded by this gene is similar to a mitochondrial di-iron containing hydroxylase in Saccharomyces cerevisiae that is involved with ubiquinone biosynthesis. Mutations in the yeast gene lead to slower development and longer life span. Alternatively spliced transcript variants have been fo und for this gene. [provided by RefSeq, Jul 2010] | 16 | 19067614 | 19083097 | Human | 192 | symbol , old_gene_name , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 8741413 | Coq7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits 3-demethoxyubiquinone 3-hydroxylase (NADH) activity (ortholog); chromatin binding (ortholog); oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen (ortholog ); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive distal hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | NW_004955442 | 3026458 | 3033582 | Chinchilla | 44 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 11700307 | COQ7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits chromatin binding (ortholog); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive dist al hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | 16 | 19229649 | 19242131 | Bonobo | 60 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 12242447 | COQ7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits chromatin binding (ortholog); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive dist al hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrion (ortholog); nucleus (ortholog) | 6 | 25912936 | 25922123 | Dog | 55 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12656994 | Coq7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits chromatin binding (ortholog); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive dist al hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | NW_004936501 | 5035986 | 5043864 | Squirrel | 60 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 14129387 | COQ7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits chromatin binding (ortholog); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive dist al hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | | | | Pig | 59 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14371622 | COQ7 | | | | | | Fly | | symbol | gene, null |
| 18675999 | COQ7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits chromatin binding (ortholog); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive dist al hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | | | | Green Monkey | 60 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 18924742 | Coq7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits 3-demethoxyubiquinone 3-hydroxylase (NADH) activity (ortholog); chromatin binding (ortholog); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinon e biosynthetic pathway; ASSOCIATED WITH autosomal recessive distal hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | | | | Naked Mole-Rat | 56 | symbol , old_gene_name | gene, protein-coding, MODEL [RefSeq] |
| 155246740 | coq7 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 626127923 | Coq7 | coenzyme Q7, hydroxylase | ENCODES a protein that exhibits 3-demethoxyubiquinone 3-hydroxylase (NADH) activity (ortholog); chromatin binding (ortholog); oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen (ortholog ); INVOLVED IN cellular response to oxidative stress (ortholog); determination of adult lifespan (ortholog); in utero embryonic development (ortholog); PARTICIPATES IN ubiquinone biosynthetic pathway; ASSOCIATED WITH autosomal recessive distal hereditary motor neuronopathy 9 (ortholog); Cachexia (ortholog); Carcinoma, Lewis Lung (ortholog); FOUND IN extrinsic component of mitochondrial inner membrane (ortholog); mitochondrial inner membrane (ortholog); mitochondrion (ortholog) | | | | Black Rat | 44 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155231442 | coq7.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 16563288 | COQ7-DT | COQ7 divergent transcript | | 16 | 19062754 | 19067691 | Human | | symbol , old_gene_name , COSMIC , name , Human Proteome Map | gene, ncrna, VALIDATED [RefSeq] |
| 1310383 | Pptc7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits protein serine/threonine phosphatase activity (ortholog); protein-macromolecule adaptor activity (ortholog); INVOLVED IN negative regulation of mitophagy (ortholog); positive regulation of ubiquinone biosynthetic process (ortholog); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrial outer membrane (ortholog); mitochondrion (ortholog); INTERACTS WITH 17beta-estradiol; 2,3,7,8-tetrachlorodibenzodioxine; 3,4-methylenedioxymethamphetamine | 12 | 39910728 | 39949101 | Rat | 109 | name | gene, protein-coding, PROVISIONAL [RefSeq] |
| 1604734 | PPTC7 | protein phosphatase targeting COQ7 | Enables protein serine/threonine phosphatase activity and protein-macromolecule adaptor activity. Involved in negative regulation of mitophagy and positive regulation of ubiquinone biosynthetic process. Located in mitochondrial matrix. Is active in mitochondrial outer membrane. [provided by Alliance of Genome Resources, Jul 2025] | 12 | 110533245 | 110583318 | Human | 105 | name | gene, protein-coding, VALIDATED [RefSeq] |
| 8963858 | Pptc7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits protein serine/threonine phosphatase activity (ortholog); protein-macromolecule adaptor activity (ortholog); INVOLVED IN negative regulation of mitophagy (ortholog); positive regulation of ubiquinone biosynthetic process (ortholog); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrial outer membrane (ortholog); mitochondrion (ortholog) | NW_004955482 | 7445218 | 7484939 | Chinchilla | 11 | name | gene, protein-coding, MODEL [RefSeq] |
| 11766407 | PPTC7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits hydrolase activity (inferred); metal ion binding (inferred); phosphoprotein phosphatase activity (inferred); INVOLVED IN negative regulation of mitophagy (inferred); positive regulation of ubiquinone biosynthetic process (inferred); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial outer membrane (inferred); mitochondrion (inferred) | 12 | 108103217 | 108152088 | Bonobo | 18 | name | gene, protein-coding, MODEL [RefSeq] |
| 12327410 | PPTC7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits hydrolase activity (inferred); metal ion binding (inferred); phosphoprotein phosphatase activity (inferred); INVOLVED IN negative regulation of mitophagy (inferred); positive regulation of ubiquinone biosynthetic process (inferred); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrion (ortholog); INTERACTS WITH bisphenol A | 26 | 8345690 | 8383902 | Dog | 20 | name | gene, protein-coding, MODEL [RefSeq] |
| 12589226 | Pptc7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits hydrolase activity (inferred); metal ion binding (inferred); phosphoprotein phosphatase activity (inferred); INVOLVED IN negative regulation of mitophagy (inferred); positive regulation of ubiquinone biosynthetic process (inferred); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial outer membrane (inferred); mitochondrion (inferred) | NW_004936558 | 3491225 | 3531886 | Squirrel | 18 | name | gene, protein-coding, MODEL [RefSeq] |
| 14229849 | PPTC7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits hydrolase activity (inferred); metal ion binding (inferred); phosphoprotein phosphatase activity (inferred); INVOLVED IN negative regulation of mitophagy (inferred); positive regulation of ubiquinone biosynthetic process (inferred); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrion (ortholog) | | | | Pig | 19 | name | gene, protein-coding, MODEL [RefSeq] |
| 18469673 | PPTC7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits hydrolase activity (inferred); metal ion binding (inferred); phosphoprotein phosphatase activity (inferred); INVOLVED IN negative regulation of mitophagy (inferred); positive regulation of ubiquinone biosynthetic process (inferred); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrial matrix (inferred); mitochondrial outer membrane (inferred); mitochondrion (inferred) | | | | Green Monkey | 18 | name | gene, protein-coding, MODEL [RefSeq] |
| 18925371 | Pptc7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits protein-macromolecule adaptor activity (ortholog); INVOLVED IN negative regulation of mitophagy (ortholog); positive regulation of ubiquinone biosynthetic process (ortholog); ASSOCIATED WITH Chemical and Drug Induced Liver Injury (ortholog); FOUND IN mitochondrial mat rix (ortholog); mitochondrial outer membrane (ortholog) | | | | Naked Mole-Rat | 16 | name | gene, protein-coding, MODEL [RefSeq] |
| 625844713 | Pptc7 | protein phosphatase targeting COQ7 | ENCODES a protein that exhibits protein serine/threonine phosphatase activity (ortholog); protein-macromolecule adaptor activity (ortholog); INVOLVED IN negative regulation of mitophagy (ortholog); positive regulation of ubiquinone biosynthetic process (ortholog); ASSOCIATED WITH Chemical and Drug I nduced Liver Injury (ortholog); FOUND IN mitochondrial matrix (ortholog); mitochondrial outer membrane (ortholog); mitochondrion (ortholog) | | | | Black Rat | 11 | name | gene, protein-coding, MODEL [RefSeq] |
| 1321260 | Pptc7 | PTC7 protein phosphatase homolog | Predicted to enable protein serine/threonine phosphatase activity. Predicted to be involved in peptidyl-serine dephosphorylation and positive regulation of ubiquinone biosynthetic process. Located in mitochondrion. Is expressed in several structures, including brain; cardiovascular system; connectiv e tissue; genitourinary system; and gut. Orthologous to human PPTC7 (protein phosphatase targeting COQ7). [provided by Alliance of Genome Resources, Apr 2025] | 5 | 122422461 | 122462344 | Mouse | 133 | description | gene, protein-coding, PROVISIONAL [RefSeq] |