Affinage

COQ7

NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase, mitochondrial · UniProt Q99807

Length
217 aa
Mass
24.3 kDa
Annotated
2026-06-09
64 papers in source corpus 22 papers cited in narrative 22 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

COQ7 (CLK-1) is the mitochondrial di-iron carboxylate hydroxylase that catalyzes a penultimate step of coenzyme Q (ubiquinone) biosynthesis, converting 5-demethoxyubiquinone (DMQ) to the hydroxylated intermediate en route to mature CoQ (PMID:8621692, PMID:11435415, PMID:20923139). Its requirement for ubiquinone synthesis is deeply conserved: yeast Coq7p, C. elegans CLK-1, and mouse COQ7 are functionally interchangeable, and loss of the enzyme in any of these systems blocks CoQ production and causes accumulation of the DMQ precursor (PMID:9020081, PMID:8621692, PMID:11244089, PMID:11716496). The protein is imported into the mitochondrial matrix and associates with the inner membrane, where it operates (PMID:9452453, PMID:11387338). Biochemically, COQ7 carries a ferritin-like di-iron carboxylate center that, with NADH serving directly as the reductant, activates dioxygen for substrate oxidation without a separate reductase, and its activity depends on active-site metal occupancy—chelation by clioquinol abolishes activity in a manner reversed by iron or cobalt (PMID:18927074, PMID:20923139). COQ7 functions in a complex with the lipid-binding protein COQ9: COQ9 presents its bound hydrophobic CoQ intermediate to the COQ7 active site, and two COQ7:COQ9 heterodimers form a curved, membrane-deforming tetramer (assembling further into a lipid-containing octamer) that extracts CoQ intermediates from the bilayer for catalysis (PMID:25339443, PMID:36306796). The enzyme is regulated post-translationally by phosphorylation, with the mitochondrial phosphatase Ptc7p dephosphorylating and thereby activating yeast Coq7p (PMID:23940037), and at the mRNA level through stabilization of COQ7 transcript by the RNA-binding protein HuR (PMID:26690054). Because CoQ is essential for respiration, loss of COQ7 produces mitochondrial respiratory failure, abnormal mitochondrial structure, and embryonic lethality in mouse, and a homozygous COQ7 missense mutation (V141E) causes primary CoQ deficiency disease in humans, rescuable by the bypass metabolite 2,4-dihydroxybenzoic acid (PMID:11716496, PMID:26084283). The pleiotropic developmental, behavioral, and lifespan phenotypes of clk-1 mutants in C. elegans are attributable to loss of ubiquinone biosynthesis (PMID:28404998).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 1996 High

    Established that COQ7 acts at a defined hydroxylase step of ubiquinone biosynthesis by showing its loss blocks CoQ and causes accumulation of the DMQ precursor.

    Evidence Genetic deletion/complementation in S. cerevisiae with HPLC lipid analysis and nonfermentable growth assays

    PMID:8621692

    Open questions at the time
    • Did not directly demonstrate enzymatic chemistry or the catalytic cofactor
    • Substrate-presentation and partner requirements unknown
  2. 1997 High

    Demonstrated deep evolutionary conservation of COQ7 function by showing C. elegans clk-1 rescues yeast coq7/cat5 nulls, unifying the gene's role across species.

    Evidence Cross-species genetic complementation and sequence homology analysis

    PMID:9020081

    Open questions at the time
    • Conservation of function did not define molecular mechanism
    • Catalytic identity still inferential
  3. 1998 High

    Resolved competing functional models by localizing Coq7p to the mitochondrial inner membrane and showing its apparent transcriptional/gluconeogenic role is secondary to respiratory failure.

    Evidence Subcellular fractionation and genetic epistasis with respiration-defective mutants

    PMID:9452453

    Open questions at the time
    • Did not establish enzymatic mechanism
    • Membrane association mode not detailed
  4. 2001 High

    Defined COQ7 as a di-iron carboxylate hydroxylase and confirmed its direct enzymatic activity through bacterial homolog complementation, while characterizing its mitochondrial import and the consequences of its loss in metazoans.

    Evidence Sequence/structural analysis with E. coli ubiB complementation; import/protease-protection assays; mouse knockout with CoQ analysis and EM; C. elegans quinone HPLC and respiratory assays

    PMID:11244089 PMID:11387338 PMID:11435415 PMID:11716496

    Open questions at the time
    • Direct iron measurement not yet performed
    • Identity of the in vivo reductant unresolved
    • How DMQ reaches the active site unknown
  5. 2003 Medium

    Confirmed that the eukaryotic CLK-1/Coq7 family directly catalyzes DMQ hydroxylation, cementing its enzymatic assignment.

    Evidence Complementation of E. coli ubiF mutant with growth and ubiquinone synthesis readouts

    PMID:12753928

    Open questions at the time
    • Single lab; no purified enzyme kinetics
    • Cofactor chemistry not directly probed
  6. 2006 Medium

    Separated COQ7's catalytic role from a structural role, showing it (with quinone lipids) stabilizes other Coq polypeptides within a high-molecular-mass biosynthetic complex independent of its hydroxylase chemistry.

    Evidence UbiF complementation, gel filtration co-migration, and immunoblotting of Coq3/Coq4/Coq6 in yeast

    PMID:16624818

    Open questions at the time
    • Stoichiometry and architecture of the complex undefined
    • Mechanism of polypeptide stabilization unclear
  7. 2010 High

    Directly proved the di-iron center and showed NADH can serve as the reductant without an accessory reductase, defining the core catalytic mechanism.

    Evidence E. coli expression, Mössbauer and EPR spectroscopy, and in vitro activity assays with quinone substrates and NADH

    PMID:20923139

    Open questions at the time
    • Physiological reductant in vivo not confirmed
    • Structural basis of substrate access not yet resolved
  8. 2013 Medium

    Identified post-translational regulation of COQ7 activity, showing phosphorylated Coq7p is inactive and the phosphatase Ptc7p activates it by dephosphorylation.

    Evidence PTC7 deletion, CoQ6 HPLC, phosphorylation analysis, and epistasis/aerobic growth assays in yeast

    PMID:23940037

    Open questions at the time
    • Phospho-sites and kinase not fully mapped
    • Conservation of phosphoregulation in mammals not established here
  9. 2014 High

    Established the molecular basis of COQ7-COQ9 partnership, showing COQ9 binds lipids/CoQ and presents substrate to COQ7 via a defined surface patch.

    Evidence Crystal structure of human COQ9, Co-IP/pulldown, MS lipid identification, mutagenesis, and a disease mouse model

    PMID:25339443

    Open questions at the time
    • Did not yet visualize the assembled COQ7:COQ9 complex
    • Mechanism of lipid handoff inferred not observed
  10. 2015 Medium

    Extended COQ7 regulation to the transcript level and linked it to human disease, while a contested report proposed a nuclear retrograde-signaling form.

    Evidence HuR RIP/mRNA stability with metabolic readouts; patient WES with 2,4-DHB bypass rescue in fibroblasts; subcellular fractionation/lifespan assays for the nuclear-CLK-1 claim

    PMID:25961505 PMID:26084283 PMID:26690054

    Open questions at the time
    • Nuclear localization was not detected in a subsequent study (PMID 28404998)
    • Generality of HuR regulation across tissues unknown
    • Single patient for the disease allele
  11. 2017 Medium

    Argued that all pleiotropic clk-1 phenotypes derive from loss of ubiquinone biosynthesis and found no nuclear enrichment, directly challenging a separate nuclear-signaling function.

    Evidence Immunohistochemistry, mammalian fractionation, pharmacological UQ restoration, and lifespan/gene-expression analysis

    PMID:28404998

    Open questions at the time
    • Single lab; reconciliation with nuclear-CLK-1 report unresolved
    • Does not exclude minor non-canonical pools
  12. 2022 High

    Provided the structural mechanism of catalysis-in-membrane, showing COQ7:COQ9 heterodimers form a curved tetramer that deforms the inner membrane to extract CoQ intermediates, and refined understanding of active-site metal selectivity.

    Evidence Cryo-EM of the COQ7:COQ9 complex with bound lipid/substrate/NADH plus molecular dynamics and mutagenesis; cell-based activity assays with manganese/cobalt metal competition

    PMID:36176269 PMID:36306796

    Open questions at the time
    • In vivo relevance of the soluble octamer not established
    • Metal-occupancy regulation (Mn/Co) rests on single low-confidence cell assay without direct occupancy measurement

Open questions

Synthesis pass · forward-looking unresolved questions
  • How COQ7 activity is integrated across phosphoregulation, metal occupancy, membrane-deformation dynamics, and tissue-specific regulation to control CoQ output in mammals remains unresolved.
  • Mammalian phosphoregulation circuitry undefined
  • In vivo physiological reductant and metal-loading control unconfirmed
  • Spectrum of human COQ7 disease alleles and genotype-phenotype relationships incomplete

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016491 oxidoreductase activity 4 GO:0016787 hydrolase activity 3
Localization
GO:0005739 mitochondrion 3
Pathway
R-HSA-1430728 Metabolism 3
Partners
COQ9ELAVL1 (HUR)PTC7
Complex memberships
COQ7:COQ9 hydroxylase complexhigh-molecular-mass CoQ biosynthetic complex (Coq3/Coq4/Coq7)

Evidence

Reading pass · 22 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1997 CLK-1 (C. elegans) is structurally and functionally conserved with yeast Cat5p/Coq7p; clk-1 complemented cat5/coq7 null mutants in yeast, demonstrating shared biochemical function in ubiquinone biosynthesis. Genetic complementation of yeast coq7/cat5 null mutants by clk-1; sequence homology analysis Science High 9020081
1996 Yeast COQ7 is required for ubiquinone biosynthesis; coq7 mutants lack ubiquinone but accumulate demethoxyubiquinone (DMQ), placing COQ7 at a hydroxylase step converting DMQ to ubiquinone. Genetic deletion and complementation in S. cerevisiae; lipid analysis by HPLC; growth assay on nonfermentable carbon sources The Journal of biological chemistry High 8621692
1998 Yeast Coq7p/Cat5p is localized to the mitochondrial inner membrane and is directly involved in ubiquinone biosynthesis; the defect in gluconeogenic gene activation in coq7/cat5 null mutants is a secondary consequence of impaired respiration, not a direct transcriptional function. Subcellular fractionation; mitochondrial localization assay; genetic epistasis with respiration-defective mutants The Journal of biological chemistry High 9452453
1999 C. elegans CLK-1 fused to GFP is fully active and localizes to mitochondria in all somatic cells; overexpression of CLK-1 increases mitochondrial activity and accelerates behavioral rates while shortening lifespan, demonstrating a regulatory role in mitochondrial function and aging. GFP fusion protein live imaging; mitochondrial dye-uptake assay in vivo; succinate cytochrome c reductase assay in vitro; transgenic overexpression The EMBO journal High 10202142
2001 CLK-1 is absolutely required for ubiquinone (UQ9) biosynthesis in C. elegans; clk-1 mutants lack UQ9 and instead accumulate DMQ9, which can partially substitute as an electron carrier in the respiratory chain. Lipid extraction and HPLC analysis of quinone species; mitochondrial respiratory enzyme activity assays (NADH-cytochrome c reductase, succinate-cytochrome c reductase); chemical synthesis of DMQ2 and functional assay The Journal of biological chemistry High 11244089
2001 COQ7 belongs to the di-iron carboxylate oxidase/hydroxylase family based on a conserved iron-ligand sequence motif; bacterial COQ7 homologs from P. aeruginosa and T. ferrooxidans complement an E. coli mutant lacking the 5-demethoxyubiquinone hydroxylase, confirming direct hydroxylase function. Sequence analysis; cloning of bacterial COQ7 homologs; functional complementation of E. coli ubiB mutant; structural modeling The Journal of biological chemistry Medium 11435415
2001 Mouse COQ7 (mCLK1) is synthesized as a preprotein, imported into the mitochondrial matrix where the leader sequence is cleaved, and becomes loosely associated with the inner membrane; unusually, this import does not require mitochondrial membrane potential. Subcellular fractionation; protease protection assay; import assay with membrane potential uncouplers; immunoblotting of processed vs. precursor forms The Journal of biological chemistry High 11387338
2001 Mouse COQ7 is essential for CoQ9 synthesis; COQ7-deficient mouse embryos fail to synthesize CoQ9 and instead accumulate DMQ9; COQ7 deficiency causes mitochondrial structural abnormalities (enlarged mitochondria with vesicular cristae) and embryonic lethality at E10.5. Targeted gene disruption (knockout mouse); biochemical CoQ analysis; electron microscopy; cell culture rescue experiments Biochemical and biophysical research communications High 11716496
2001 The maternal rescue phenotype of C. elegans clk-1 mutants is explained by persistence of small amounts of maternally provided CLK-1 protein, which is sufficient for synthesis of considerable ubiquinone during early development; gradual depletion of CLK-1 and ubiquinone drives expression of mutant phenotype. Western blotting for CLK-1 protein levels; ubiquinone measurement; developmental arrest experiments; double mutant analysis (daf-2 clk-1) The Journal of biological chemistry Medium 14517217
2002 C. elegans CLK-1 and mouse CLK-1 have DNA-binding activity specific to the OL (light-strand origin of replication) region of mitochondrial DNA; this activity is inhibited by ADP and is altered by life-span-extending clk-1 mutations. Electrophoretic mobility shift assay (EMSA); competition binding with specific vs. nonspecific DNA; ADP inhibition assay; mutant vs. wild-type protein comparison FEBS letters Low 11959146
2003 C. elegans CLK-1 functions as a demethoxyubiquinone (DMQ) hydroxylase; complementation of E. coli ubiF mutants by C. elegans CLK-1 demonstrated that the eukaryotic CLK-1/Coq7 family can directly catalyze the hydroxylation of DMQ to produce hydroxyubiquinone. Functional complementation of E. coli ubiF mutant; growth on nonfermentable carbon source; ubiquinone synthesis assay FEBS letters Medium 12753928
2006 Yeast Coq7p co-migrates with Coq3p and Coq4p as a high-molecular-mass complex; Coq7p (and the quinone lipids DMQ and Q) function to stabilize other Coq polypeptides; E. coli UbiF can substitute for the hydroxylase activity of Coq7p but the structural/stabilization role requires Coq7p itself. Complementation of coq7 point and null mutants by UbiF; gel filtration co-migration analysis; immunoblotting for Coq3, Coq4, Coq6 levels; addition of exogenous Q to null mutant The Journal of biological chemistry Medium 16624818
2008 Clioquinol (a metal chelator) inhibits mammalian CLK-1 (COQ7) enzymatic activity in cultured cells; this inhibition is blocked by iron or cobalt cations, indicating chelation of the active-site metal is the mechanism of action. Cell-based CLK-1 activity assay; metal cation rescue experiments (iron, cobalt); phenotypic comparison of clioquinol-treated vs. clk-1 mutant nematodes and mice The Journal of biological chemistry Medium 18927074
2010 Mouse CLK-1 (MCLK1) is a di-iron carboxylate protein; Mössbauer and EPR spectroscopy confirmed iron binding; in vitro activity assays showed NADH can serve directly as reductant for the diiron center to activate dioxygen and catalyze substrate oxidation, with no requirement for an additional reductase protein. Heterologous expression in E. coli; Mössbauer spectroscopy; EPR spectroscopy; in vitro enzyme activity assay with quinone substrates and NADH Biochemistry High 20923139
2013 The mitochondrial Ser/Thr phosphatase Ptc7p activates CoQ6 biosynthesis in yeast by dephosphorylating Coq7p; phosphorylated Coq7p is inactive, and Ptc7p-mediated dephosphorylation is required for Coq7p hydroxylase activity. Genetic deletion of PTC7; CoQ6 measurement by HPLC; phosphorylation analysis; epistasis experiments; aerobic growth assays The Journal of biological chemistry Medium 23940037
2014 COQ9 specifically interacts with COQ7 through conserved residues in mammals; COQ9 has a lipid-binding site and associates with multiple lipid species including CoQ itself; the COQ9 residues necessary for COQ7 interaction form a surface patch around the lipid-binding site, suggesting COQ9 presents its bound lipid substrate to COQ7. Crystal structure of human COQ9 at 2.4 Å; Co-IP/pulldown of COQ9-COQ7 interaction; MS-based lipid identification; disease-associated mouse model with COQ9 mutation showing disrupted CoQ protein complex; mutagenesis of conserved residues Proceedings of the National Academy of Sciences of the United States of America High 25339443
2015 A nuclear form of CLK-1 exists in C. elegans (and is conserved to humans) that independently regulates lifespan through a retrograde signaling pathway responsive to mitochondrial ROS; nuclear CLK-1 modulates gene expression to regulate mitochondrial ROS metabolism and the mitochondrial unfolded protein response. Subcellular fractionation; fluorescence imaging of tagged CLK-1; genetic epistasis with ROS pathway mutants; gene expression analysis; lifespan assays with nuclear-localized CLK-1 constructs Nature cell biology Medium 25961505
2015 The RNA-binding protein HuR stabilizes COQ7 mRNA via binding to the 3'-UTR (first 765 bp); HuR knockdown or serum deprivation destabilizes COQ7 mRNA, reduces COQ7 protein levels, decreases CoQ biosynthesis, reduces oxygen consumption and ATP production, and increases glycolysis. RNA-binding protein pulldown (RIP); mRNA stability assay (half-life measurement); siRNA knockdown of HuR and hnRNP C1/C2; oxygen consumption rate (Seahorse); ATP and lactate measurements RNA biology Medium 26690054
2015 A homozygous COQ7 missense mutation (V141E) in a human patient causes primary CoQ deficiency; 2,4-dihydroxybenzoic acid (2,4-DHB), which bypasses the COQ7 enzymatic step, rescues CoQ levels and biochemical defects in patient fibroblasts. Whole exome sequencing; CoQ measurement by UPLC-MS in patient fibroblasts; rescue with 2,4-DHB; functional mitochondrial assays Journal of medical genetics Medium 26084283
2017 All pleiotropic phenotypes of C. elegans clk-1 null mutants (behavioral, developmental, mitochondrial quality control gene expression, lifespan) are attributable to loss of ubiquinone biosynthesis; pharmacological restoration of UQ biosynthesis rescues all phenotypes; no nuclear enrichment of MCLK1 or CLK-1 was detected in worms or mammalian cell fractionation. Immunohistochemistry in C. elegans; subcellular fractionation in mammalian cells; pharmacological UQ restoration; lifespan analysis; gene expression analysis; CLK-1 constructs lacking mitochondrial targeting sequence Scientific reports Medium 28404998
2022 COQ7 adopts a ferritin-like (di-iron carboxylate) fold with a hydrophobic channel whose substrate-binding capacity is enhanced by COQ9; two COQ7:COQ9 heterodimers form a curved tetramer that deforms the mitochondrial inner membrane, potentially allowing CoQ intermediates to translocate from the bilayer to the proteins' lipid-binding sites; two tetramers assemble into a soluble octamer with a pseudo-bilayer of lipids captured within. Cryo-EM structure of COQ7:COQ9 complex with bound lipid/substrate/NADH; molecular dynamics simulations; structure-function mutagenesis Molecular cell High 36306796
2022 Manganese exposure inhibits COQ7 di-iron carboxylate hydroxylase activity in mouse cells; pre-treatment with cobalt interferes with manganese inhibition, suggesting cobalt has greater affinity than both iron and manganese for the COQ7 active site and maintains catalytic activity when substituted. Cell-based COQ7 activity assay (CoQ biosynthesis measurement); manganese and cobalt treatment; competitive metal replacement assay microPublication biology Low 36176269

Source papers

Stage 0 corpus · 64 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1995 Mutations in the clk-1 gene of Caenorhabditis elegans affect developmental and behavioral timing. Genetics 348 7768437
2005 Evolutionary conservation of the clk-1-dependent mechanism of longevity: loss of mclk1 increases cellular fitness and lifespan in mice. Genes & development 291 16195414
1997 Structural and functional conservation of the Caenorhabditis elegans timing gene clk-1. Science (New York, N.Y.) 255 9020081
1999 CLK-1 controls respiration, behavior and aging in the nematode Caenorhabditis elegans. The EMBO journal 235 10202142
1999 A cytosolic catalase is needed to extend adult lifespan in C. elegans daf-C and clk-1 mutants. Nature 191 10335847
2001 Altered quinone biosynthesis in the long-lived clk-1 mutants of Caenorhabditis elegans. The Journal of biological chemistry 168 11244089
1996 The COQ7 gene encodes a protein in saccharomyces cerevisiae necessary for ubiquinone biosynthesis. The Journal of biological chemistry 157 8621692
2001 A dietary source of coenzyme Q is essential for growth of long-lived Caenorhabditis elegans clk-1 mutants. Proceedings of the National Academy of Sciences of the United States of America 155 11136229
1998 Yeast Clk-1 homologue (Coq7/Cat5) is a mitochondrial protein in coenzyme Q synthesis. The Journal of biological chemistry 110 9452453
2001 A new member of the family of di-iron carboxylate proteins. Coq7 (clk-1), a membrane-bound hydroxylase involved in ubiquinone biosynthesis. The Journal of biological chemistry 108 11435415
2014 Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis. Proceedings of the National Academy of Sciences of the United States of America 104 25339443
2015 Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4-dihydroxybensoic acid. Journal of medical genetics 95 26084283
2015 A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity. Nature cell biology 76 25961505
2001 Mouse homologue of coq7/clk-1, longevity gene in Caenorhabditis elegans, is essential for coenzyme Q synthesis, maintenance of mitochondrial integrity, and neurogenesis. Biochemical and biophysical research communications 71 11716496
2000 clk-1, mitochondria, and physiological rates. BioEssays : news and reviews in molecular, cellular and developmental biology 66 10649290
1995 CAT5, a new gene necessary for derepression of gluconeogenic enzymes in Saccharomyces cerevisiae. The EMBO journal 59 8557031
2017 Pathogenicity of two COQ7 mutations and responses to 2,4-dihydroxybenzoate bypass treatment. Journal of cellular and molecular medicine 57 28409910
1999 Conservation of the Caenorhabditis elegans timing gene clk-1 from yeast to human: a gene required for ubiquinone biosynthesis with potential implications for aging. Mammalian genome : official journal of the International Mammalian Genome Society 56 10501970
2006 Complementation of Saccharomyces cerevisiae coq7 mutants by mitochondrial targeting of the Escherichia coli UbiF polypeptide: two functions of yeast Coq7 polypeptide in coenzyme Q biosynthesis. The Journal of biological chemistry 51 16624818
2008 The anti-neurodegeneration drug clioquinol inhibits the aging-associated protein CLK-1. The Journal of biological chemistry 48 18927074
2013 TAF-4 is required for the life extension of isp-1, clk-1 and tpk-1 Mit mutants. Aging 42 24107417
2013 The phosphatase Ptc7 induces coenzyme Q biosynthesis by activating the hydroxylase Coq7 in yeast. The Journal of biological chemistry 41 23940037
2001 Phenotypic and suppressor analysis of defecation in clk-1 mutants reveals that reaction to changes in temperature is an active process in Caenorhabditis elegans. Genetics 39 11729148
1996 Isolation and sequencing of the rat Coq7 gene and the mapping of mouse Coq7 to chromosome 7. Archives of biochemistry and biophysics 38 8660658
2008 Comparison of a coq7 deletion mutant with other respiration-defective mutants in fission yeast. The FEBS journal 35 18808426
2022 Structure and functionality of a multimeric human COQ7:COQ9 complex. Molecular cell 34 36306796
2019 A fatal case of COQ7-associated primary coenzyme Q10 deficiency. JIMD reports 34 31240163
2001 Mouse CLK-1 is imported into mitochondria by an unusual process that requires a leader sequence but no membrane potential. The Journal of biological chemistry 33 11387338
2010 The aging-associated enzyme CLK-1 is a member of the carboxylate-bridged diiron family of proteins. Biochemistry 31 20923139
2004 coq7/clk-1 regulates mitochondrial respiration and the generation of reactive oxygen species via coenzyme Q. Aging cell 30 15379851
2015 RNA-binding proteins regulate cell respiration and coenzyme Q biosynthesis by post-transcriptional regulation of COQ7. RNA biology 28 26690054
2006 Genetic and molecular characterization of CLK-1/mCLK1, a conserved determinant of the rate of aging. Experimental gerontology 28 16889924
2003 Sensitivity of Caenorhabditis elegans clk-1 mutants to ubiquinone side-chain length reveals multiple ubiquinone-dependent processes. The Journal of biological chemistry 28 12893826
2017 A single biochemical activity underlies the pleiotropy of the aging-related protein CLK-1. Scientific reports 26 28404998
1999 Orthologues of the Caenorhabditis elegans longevity gene clk-1 in mouse and human. Genomics 25 10373327
2006 Uncoupling the pleiotropic phenotypes of clk-1 with tRNA missense suppressors in Caenorhabditis elegans. Molecular and cellular biology 24 16648490
2003 Molecular mechanism of maternal rescue in the clk-1 mutants of Caenorhabditis elegans. The Journal of biological chemistry 24 14517217
2001 CLK-1/Coq7p is a DMQ mono-oxygenase and a new member of the di-iron carboxylate protein family. FEBS letters 24 11749961
2023 Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy. Brain : a journal of neurology 22 36454683
2022 A novel COQ7 mutation causing primarily neuromuscular pathology and its treatment options. Molecular genetics and metabolism reports 22 35782625
2008 Clk-1 deficiency induces apoptosis associated with mitochondrial dysfunction in mouse embryos. Mechanisms of ageing and development 17 18343482
2023 Biallelic variants in COQ7 cause distal hereditary motor neuropathy with upper motor neuron signs. Brain : a journal of neurology 16 37170631
2001 Mouse coq7/clk-1 orthologue rescued slowed rhythmic behavior and extended life span of clk-1 longevity mutant in Caenorhabditis elegans. Biochemical and biophysical research communications 13 11511092
2024 COQ7 defect causes prenatal onset of mitochondrial CoQ10 deficiency with cardiomyopathy and gastrointestinal obstruction. European journal of human genetics : EJHG 11 38702428
2002 CLK-1 protein has DNA binding activity specific to O(L) region of mitochondrial DNA. FEBS letters 11 11959146
2023 Novel Homozygous Variant in COQ7 in Siblings With Hereditary Motor Neuropathy. Neurology. Genetics 10 37077559
2023 Phenotypic, molecular, and functional characterization of COQ7-related primary CoQ10 deficiency: Hypomorphic variants and two distinct disease entities. Molecular genetics and metabolism 10 37392700
2012 Restoration of the behavioral rates and lifespan in clk-1 mutant nematodes in response to exogenous coenzyme Q(10). Experimental gerontology 10 22244837
2021 Elevated mitochondrial DNA copy number found in ubiquinone-deficient clk-1 mutants is not rescued by ubiquinone precursor 2-4-dihydroxybenzoate. Mitochondrion 8 33581333
2014 Extended lifespan, reduced body size and leg skeletal muscle mass, and decreased mitochondrial function in clk-1 transgenic mice. Experimental gerontology 8 25106098
2024 New variants expand the neurological phenotype of COQ7 deficiency. Journal of inherited metabolic disease 7 38973597
2003 Complementation of Escherichia coli ubiF mutation by Caenorhabditis elegans CLK-1, a product of the longevity gene of the nematode worm. FEBS letters 7 12753928
2019 Identification of small molecule inhibitors of human COQ7. Bioorganic & medicinal chemistry 5 31753803
2012 Reversal of slow growth and heartbeat through the restoration of mitochondrial function in clk-1-deficient mouse embryos by exogenous administration of coenzyme Q10. Experimental gerontology 5 22465812
2024 Homozygous variant in COQ7 causes autosomal recessive hereditary spastic paraplegia. Annals of clinical and translational neurology 4 38439593
2023 SGC-CLK-1: A chemical probe for the Cdc2-like kinases CLK1, CLK2, and CLK4. Current research in chemical biology 4 38009092
2018 Ethanol's Effect on Coq7 Expression in the Hippocampus of Mice. Frontiers in genetics 4 30564271
2016 CLD1 Reverses the Ubiquinone Insufficiency of Mutant cat5/coq7 in a Saccharomyces cerevisiae Model System. PloS one 3 27603010
2022 The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt. microPublication biology 2 36176269
2023 A non-canonical Puf3p-binding sequence regulates CAT5/COQ7 mRNA under both fermentable and respiratory conditions in budding yeast. PloS one 1 38100455
2026 Nematode-trapping fungus Arthrobotrys oligospora is hungry for iron-chelating agent COQ7 of nematodes. Natural products and bioprospecting 0 41770440
2026 COQ7-Related Neuropathy: Two New Cases and Review of the Literature. Journal of the peripheral nervous system : JPNS 0 42240099
2025 Stress responses and dynamic equilibrium: Key determinants of aging in the C. elegans clk-1 mutant. bioRxiv : the preprint server for biology 0 40949981
2024 COQ7 splice site variant causing a spastic paraparesis phenotype in siblings. Journal of genetics 0 39080983

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