Affinage

COQ3

Ubiquinone biosynthesis O-methyltransferase, mitochondrial · UniProt Q9NZJ6

Length
369 aa
Mass
41.1 kDa
Annotated
2026-06-09
57 papers in source corpus 20 papers cited in narrative 20 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

COQ3 encodes a S-adenosylmethionine-dependent O-methyltransferase that catalyzes both O-methylation steps of the coenzyme Q biosynthetic pathway, a function conserved from the E. coli ortholog UbiG through yeast, rat, and human enzymes (PMID:1885593, PMID:8703953, PMID:10777520). The yeast and rat enzymes O-methylate both early (3,4-dihydroxy-5-polyprenylbenzoate) and late (demethyl-Q) CoQ intermediates, and the human enzyme is active on all corresponding substrates in vitro (PMID:10419476, PMID:10777520). The protein functions in the mitochondrial matrix, where it is peripherally associated with the matrix face of the inner membrane, and mitochondrial targeting is essential for activity in vivo (PMID:10419476, PMID:24911838). COQ3/UbiG defines a class of cardiolipin-binding methyltransferases: it adopts a Class I SAM-methyltransferase fold with a conserved insertion that mediates preferential binding to cardiolipin-enriched membranes and is required for function (PMID:26251450). COQ3 is a core catalytic subunit of a high-molecular-mass (~1 MDa) multi-subunit CoQ biosynthetic complex (the CoQ synthome/metabolon, containing Coq3–Coq9), whose assembly and the steady-state stability of Coq3 depend on the other Coq subunits and on a polyprenyl/Q-derived lipid intermediate (PMID:10760477, PMID:17391640, PMID:24406904, PMID:38425362). The animal metabolon comprising COQ3–COQ9 has been reconstituted in vitro, confirming COQ3's position as a core enzyme within it (PMID:38425362). The mitochondrial NADPH oxidoreductase RTN4IP1 regulates COQ3 O-methylation activity in muscle (PMID:37884807). The ubiquinol generated downstream of COQ3 acts as a lipid-soluble antioxidant that protects against lipid peroxidation in vivo (PMID:8755509).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 1991 High

    Established the molecular identity of COQ3 by showing it encodes the DHHB methyltransferase required for CoQ biosynthesis, linking a genetic locus to an enzymatic step.

    Evidence Functional complementation of a yeast coq3 mutant with chromosomal gene replacement and sequence analysis revealing methyltransferase consensus and homology to E. coli UbiG

    PMID:1885593

    Open questions at the time
    • Did not establish whether Coq3 catalyzes one or both O-methylation steps
    • Subcellular localization not determined
  2. 1994 High

    Demonstrated functional conservation of the methyltransferase from yeast to mammals, showing the enzyme is broadly required across eukaryotes.

    Evidence Functional complementation of a yeast coq3 deletion with a rat cDNA and sequence comparison

    PMID:8125303

    Open questions at the time
    • Did not test the mammalian enzyme's substrate range directly
    • No localization data for the mammalian protein
  3. 1999 High

    Resolved which methylation steps the enzyme performs and where it acts, showing Coq3 catalyzes both O-methylation steps and is matrix-facing on the inner membrane.

    Evidence In vitro methyltransferase assays with synthetic demethyl-Q3 and Q3 substrates across yeast, rat, and E. coli proteins, plus antibody submitochondrial localization (building on 1996 import/complementation data)

    PMID:10419476 PMID:8703953

    Open questions at the time
    • Mechanism of peripheral membrane association not defined
    • Did not identify other proteins required for in vivo activity
  4. 2000 High

    Extended dual O-methyltransferase activity to the human enzyme and revealed that Coq3 stability depends on a multi-subunit context, the first evidence for a biosynthetic complex.

    Evidence Human COQ3 complementation of yeast nulls with in vitro activity on three substrates, plus systematic enzymatic/protein analysis across a full coq null panel with RNA controls showing post-transcriptional destabilization of Coq3

    PMID:10760477 PMID:10777520

    Open questions at the time
    • Did not directly demonstrate physical complex assembly
    • Stoichiometry and architecture unknown
  5. 2004 Medium

    Identified a lipid requirement for Coq3 stability, showing the polyprenyl product of Coq1 or a Q-intermediate is needed to maintain the protein.

    Evidence Western analysis of Coq3 levels in deltacoq1 mutants complemented with diverse Coq1 orthologs producing distinct Q isoforms

    PMID:15548532

    Open questions at the time
    • Direct lipid binding to Coq3 not demonstrated here
    • Single lab; mechanism of stabilization unresolved
  6. 2007 Medium

    Provided direct biochemical evidence that Coq3 is a subunit of a discrete ~1 MDa biosynthetic complex, moving from genetic inference to physical assembly.

    Evidence Blue Native-PAGE co-migration of Coq3 with Coq4/Coq9 and co-immunoprecipitation of multiple Coq polypeptides via HA-tagged Coq9

    PMID:17391640

    Open questions at the time
    • Direct Coq3-Coq9 binary interaction not shown
    • Complex stoichiometry and assembly order undefined
  7. 2014 Medium

    Defined the complex as the CoQ synthome organized around Coq4 and showed Coq8 overexpression and Q intermediates promote its assembly, clarifying complex dynamics.

    Evidence 2D BN/SDS-PAGE of digitonin mitochondrial extracts with Coq8 overexpression in multiple coq null backgrounds and exogenous Q6 supplementation

    PMID:24406904

    Open questions at the time
    • Precise role of Coq3 in complex nucleation not isolated
    • Single lab
  8. 2015 High

    Explained how Coq3 associates with membranes by solving the UbiG structure and identifying a conserved insertion mediating cardiolipin binding required for function.

    Evidence 2.1 Å crystal structure of E. coli UbiG, liposome binding assays for human/yeast/E. coli proteins, mutagenesis abolishing binding and in vivo rescue

    PMID:26251450

    Open questions at the time
    • No structure of the eukaryotic Coq3 protein itself
    • Membrane-binding role within the assembled synthome not directly tested
  9. 2020 Medium

    Established a hierarchical interdependence in human mitochondria, placing COQ3 (with PDSS2) as a particularly important node for stabilizing other COQ proteins.

    Evidence Reciprocal siRNA knockdown with Western blot and native-gel detection of COQ3-containing complexes in human 143B cells

    PMID:32194061

    Open questions at the time
    • Direct binary interactions not mapped
    • Functional significance of multiple COQ3 isoforms unresolved
  10. 2023 High

    Identified RTN4IP1 as a regulator of COQ3 O-methylation activity in muscle, adding a tissue-specific regulatory input to the enzyme.

    Evidence Matrix-targeted APEX proximity proteomics in transgenic mice, in vitro enzymatic assays, and Rtn4ip1-KO myoblast CoQ measurement

    PMID:37884807

    Open questions at the time
    • Molecular mechanism by which RTN4IP1 modulates COQ3 activity unresolved
    • Generality beyond muscle tissue unclear
  11. 2024 High

    Reconstituted the animal COQ metabolon in vitro, biochemically confirming COQ3 as a core enzyme of the COQ3–COQ9 assembly and the streamlining role of COQ8.

    Evidence In vitro reconstitution using ancestral sequence reconstruction with enzymatic activity assays

    PMID:38425362

    Open questions at the time
    • Structure of the assembled metabolon not determined
    • Regulatory inputs in the cellular context not captured by reconstitution
  12. 2025 Medium

    Connected synthome integrity to ER–mitochondria contact sites, showing ERMES loss destabilizes the Coq3-containing complex and COQ11 deletion can repair it.

    Evidence Genetic epistasis in yeast ERMES mutants, 2D BN/SDS-PAGE of the synthome, and Split-MAM artificial tether experiments with CoQ measurement

    PMID:39906518

    Open questions at the time
    • Mechanism linking contact sites to synthome stability undefined
    • Direct effect on Coq3 specifically not isolated

Open questions

Synthesis pass · forward-looking unresolved questions
  • How COQ3 activity, membrane association, and synthome assembly are coordinated and regulated in human tissues, and the structural basis of the eukaryotic enzyme within the metabolon, remain open.
  • No structure of human/eukaryotic Coq3 protein
  • Disease-causing human COQ3 mutations not documented in this corpus
  • Mechanism of RTN4IP1 and ERMES regulation unresolved

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016740 transferase activity 4 GO:0008289 lipid binding 1
Localization
GO:0005739 mitochondrion 4
Pathway
R-HSA-1430728 Metabolism 4
Partners
ADCK3COQ4COQ5COQ6COQ7COQ9PDSS2RTN4IP1
Complex memberships
CoQ synthome / COQ metabolon (Coq3–Coq9)

Evidence

Reading pass · 20 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1991 The COQ3 gene in S. cerevisiae encodes the 3,4-dihydroxy-5-hexaprenylbenzoate (DHHB) methyltransferase, established by functional complementation of a coq3 mutant and one-step in situ gene replacement directing integration to the COQ3 locus on chromosome XV. The predicted amino acid sequence contains a methyltransferase consensus sequence and shows 40% identity with E. coli UbiG (gyrA5' open reading frame). Functional complementation of yeast coq3 mutant, in situ gene replacement, sequence analysis The Journal of biological chemistry High 1885593
1994 A rat cDNA homologue of COQ3 was isolated by functional complementation of a yeast coq3 deletion mutant, establishing that the mammalian Coq3 protein is a dihydroxypolyprenylbenzoate methyltransferase conserved from yeast to mammals. The rat sequence shares 39% identity with yeast Coq3 and 37% with E. coli UbiG over 138 amino acids. Functional complementation of yeast coq3 deletion mutant with rat cDNA library, sequence analysis Gene High 8125303
1996 E. coli UbiG catalyzes both O-methylation steps in ubiquinone biosynthesis (not just the second step). When engineered with a mitochondrial leader sequence, UbiG rescues respiration in yeast coq3 mutants. In vitro methyltransferase assays with synthetically prepared farnesylated substrate analogs showed UbiG methylates both the eukaryotic intermediate 3,4-dihydroxy-5-farnesylbenzoate and the E. coli intermediate 2-farnesyl-6-hydroxyphenol. Yeast Coq3p is located in mitochondria and mitochondrial targeting is essential for function in vivo. Mitochondrial import of Coq3p requires a membrane potential. Functional complementation, in vitro methyltransferase assays with synthetic substrate analogs, in vitro mitochondrial import assays Biochemistry High 8703953
1999 Both yeast Coq3p and rat Coq3p catalyze both O-methylation steps in coenzyme Q biosynthesis, including methylation of demethyl-Q(3) (the second O-methylation step). E. coli UbiG was purified and shown to catalyze both O-methylation steps. Coq3p is peripherally associated with the matrix-side of the inner membrane of yeast mitochondria (confirmed by antibody localization studies). Q biosynthesis occurs within the matrix compartment of yeast mitochondria. In vitro methyltransferase assays with chemically synthesized farnesylated substrate analogs (demethyl-Q3 and Q3), antibody-based submitochondrial localization studies, protein purification The Journal of biological chemistry High 10419476
2000 Human COQ3 encodes a functional O-methyltransferase required for ubiquinone biosynthesis. The human enzyme expressed in multicopy rescues yeast coq3 null mutants for growth on nonfermentable carbon sources and restores CoQ biosynthesis. In vitro methyltransferase assays demonstrated human Coq3 is active with all three substrates tested (3,4-dihydroxy-5-polyprenylbenzoic acid, demethyl-Q, and 2-hydroxy-6-polyprenyl phenol). The human protein shares 87% identity with rat Coq3 and 35% with yeast Coq3 in the conserved region. Functional complementation of yeast coq3 null mutant, in vitro methyltransferase assays with farnesylated analogs of CoQ intermediates The Journal of biological chemistry High 10777520
2000 COQ3-encoded O-methyltransferase activity and steady-state Coq3 polypeptide levels depend on the presence of the other COQ gene products (Coq1–Coq8). COQ3 mRNA levels are not decreased in coq mutants, suggesting post-transcriptional regulation (decreased translation or decreased Coq3p stability). This constitutes genetic evidence that Coq polypeptides participate in a multi-subunit complex in which absence of any one member destabilizes Coq3p. In vitro O-methyltransferase activity assays on isolated mitochondria from a complete panel of yeast coq null mutants; steady-state RNA and protein level analysis Biochimica et biophysica acta High 10760477
2003 Coq5p is required for stability of Coq3p and Coq4p. In coq5 null mutants and certain coq5 missense mutants, Coq3p and Coq4p are undetectable, establishing that Coq5p is required to maintain steady-state levels of Coq3p within the multi-subunit Q biosynthetic complex. Western blot analysis of steady-state Coq3p levels in yeast coq5 mutant collection; phenotypic complementation assays The Journal of biological chemistry Medium 14701817
2004 Coq3p levels are dependent on expression of COQ1 (hexaprenyl diphosphate synthase) and are rescued by diverse Coq1 orthologs that produce distinct isoforms of Q. Coq1p is peripherally associated with the inner membrane on the matrix side. The lipid product of Coq1p or a Q-intermediate derived from polyprenyl diphosphate is required to stabilize Coq3p. Western blot analysis of Coq3p steady-state levels in yeast deltacoq1 mutants complemented with diverse prokaryotic Coq1 orthologs; mitochondrial fractionation The Journal of biological chemistry Medium 15548532
2007 Coq3p is a subunit of a high molecular mass (~1 MDa) mitochondrial coenzyme Q biosynthetic complex. By Blue Native-PAGE, Coq3p co-migrates with Coq4p and Coq9p at ~1 MDa. Coq9p immunoprecipitates with Coq4p, Coq5p, Coq6p, and Coq7p, establishing at least six Coq polypeptides in a multi-subunit Q biosynthetic complex. Blue Native-PAGE, co-immunoprecipitation of HA-tagged Coq9p, submitochondrial fractionation Archives of biochemistry and biophysics Medium 17391640
2012 Mclk1(+/-) (Coq7 heterozygous) mice show decreased ubiquinone in the inner mitochondrial membrane with compensatory increased ubiquinone in the outer membrane. In contrast, Coq3(+/-) mice have normal lifespan and no detectable defects in mitochondrial function or ubiquinone distribution, establishing that heterozygous Coq3 deficiency does not reproduce the Mclk1 phenotype. Homozygous Coq3 knockout is embryonic lethal, as is homozygous Mclk1 null. Dietary Q10 supplementation reversed mutant mitochondrial phenotypes in Mclk1(+/-) mice. Submitochondrial fractionation of highly purified mitochondrial membranes; dietary Q10 supplementation rescue; genetic comparison of Mclk1(+/-) vs Coq3(+/-) mice The Journal of cell biology Medium 23045551
2014 Coq3p is a component of the CoQ-synthome (high molecular mass multi-subunit complex). Over-expression of Coq8 in coq3, coq5, coq6, coq7, coq9, and coq10 null mutants promotes association of Coq4 and other Coq polypeptides in high molecular mass complexes as shown by 2D BN/SDS-PAGE. Coq4 is identified as a central organizer of the Coq complex. Exogenous Q6 supplementation stabilizes Coq4, Coq7, and Coq9, and promotes late-stage Q-intermediate formation, with this stabilizing effect requiring Coq1 and Coq2 production of a polyisoprenyl intermediate. 2D blue-native/SDS-PAGE of digitonin extracts from mitochondria; exogenous Q6 supplementation; genetic epistasis with Coq8 over-expression in multiple coq null backgrounds Biochimica et biophysica acta Medium 24406904
2015 UbiG/Coq3 proteins define a novel class of membrane-binding proteins. E. coli UbiG binds specifically to liposomes containing phosphatidylglycerol (PG) or cardiolipin (CL). Human and yeast Coq3 display strong preference for liposomes enriched with cardiolipin, the signature lipid of the mitochondrial membrane. The crystal structure of E. coli UbiG was solved at 2.1 Å resolution, revealing a Class I SAM-methyltransferase fold with a unique insertion between strand β5 and helix α10 that is highly conserved and required for membrane binding. Mutagenesis of key residues in this insertion abolished liposome binding in vitro and failed to rescue the ΔubiG phenotype in vivo. Crystal structure determination (2.1 Å), liposome binding assays, site-directed mutagenesis, in vivo complementation assays The Biochemical journal High 26251450
2016 Human ADCK3 (an atypical kinase involved in CoQ10 biosynthesis) associates in vitro with recombinant Coq3, Coq5, Coq7, and Coq9 proteins, placing Coq3 as a binding partner of the ADCK3 regulatory kinase within the CoQ biosynthetic machinery. In vitro protein-protein interaction assay (recombinant protein pulldown) PloS one Low 26866375
2020 In human 143B mitochondria, PDSS2 and COQ3 play more important roles in maintaining the stability of other COQ proteins than other COQ subunits. COQ3 was detected in protein complexes in the mitochondria, including complexes containing PDSS2, COQ4, COQ6, and COQ7. Multiple isoforms of COQ3 protein were identified. Knockdown of PDSS2 suppressed COQ3 levels, while COQ3 knockdown suppressed levels of other COQ proteins. Immunoprecipitation/native gel electrophoresis of mitochondrial complexes; siRNA knockdown with Western blot; specific antibody characterization; mitochondrial localization of mature proteins Biochimica et biophysica acta. Bioenergetics Medium 32194061
2023 RTN4IP1 (OPA10), an NADPH oxidoreductase enriched in the mitochondrial matrix of muscle tissues, regulates the O-methylation activity of COQ3. Interactome analysis showed RTN4IP1 associates with COQ3. In vitro enzymatic assays demonstrated an essential role for RTN4IP1 in CoQ biosynthesis through regulation of COQ3 O-methylation activity. Rtn4ip1-knockout myoblasts showed markedly decreased CoQ9 levels and impaired cellular respiration. Proximity-labeling proteomics (matrix-targeted APEX in transgenic mice), interactome analysis, in vitro enzymatic assays, Rtn4ip1-KO myoblast CoQ measurement, muscle-specific RNAi in Drosophila Nature chemical biology High 37884807
2024 COQ3, COQ4, COQ5, COQ6, COQ7, and COQ9 form the COQ metabolon in animals, and this metabolon was reconstituted in vitro using ancestral sequence reconstruction. Within the metabolon, COQ3 participates as one of the core biosynthetic enzymes. COQ8 (a kinase) increases and streamlines coenzyme Q production when added to the in vitro reconstituted metabolon. In vitro reconstitution of the entire COQ metabolon using ancestral sequence reconstruction; enzymatic activity assays Nature catalysis High 38425362
2025 In yeast, COQ11 deletion suppresses respiratory deficiency of select ERMES (ER-mitochondria encounter structure) mutants and repairs/reorganizes the CoQ synthome (which contains Coq3-Coq9). Loss of ERMES destabilizes the CoQ synthome, implicating ER-mitochondrial contact sites in regulating CoQ biosynthesis and the stability of the complex containing Coq3. Artificial ER-mitochondria tethers (Split-MAM) influence CoQ content and turnover. Genetic epistasis (COQ11 deletion in ERMES mutant backgrounds), 2D BN/SDS-PAGE of CoQ synthome, Split-MAM artificial tether experiments, CoQ content measurement Contact (Thousand Oaks) Medium 39906518
1996 Q-deficient yeast harboring a COQ3 deletion are hypersensitive to autoxidation products of polyunsaturated fatty acids and accumulate elevated lipid hydroperoxides. This phenotype is rescued by the COQ3 gene on a single-copy plasmid, by butylated hydroxytoluene, alpha-tocopherol, or trolox (vitamin E analog), establishing that ubiquinol (QH2, the reduced form of CoQ produced via the Coq3-dependent pathway) functions as a lipid-soluble antioxidant in vivo. Genetic deletion of COQ3, lipid hydroperoxide measurement, rescue by plasmid-borne COQ3 and chemical antioxidants Proceedings of the National Academy of Sciences of the United States of America High 8755509
1998 Coenzyme Q produced by the Coq3-dependent biosynthetic pathway is required for NADH-ascorbate free radical reductase activity in the plasma membrane of S. cerevisiae. Plasma membranes from coq3Δ cells are completely devoid of CoQ6 and have ~10% of wild-type NADH-ascorbate free radical reductase activity, which is rescued by transformation with a COQ3 plasmid or growth in the presence of exogenous CoQ6. Enzyme activity assays on plasma membranes from coq3Δ cells; genetic rescue with COQ3 plasmid; exogenous Q6 supplementation rescue; comparison with respiratory-deficient but Q-replete controls (atp2Δ, cor1Δ) Journal of bioenergetics and biomembranes Medium 9932649
2014 Human COQ3 functionally complements S. pombe coq3 deletion strains and restores CoQ10 production, but only when a mitochondrial targeting sequence is added to the human construct. This establishes that the human COQ3 protein requires mitochondrial targeting for function in fission yeast, consistent with its role as a mitochondrial matrix enzyme. Functional complementation of S. pombe coq3 deletion with human COQ3 constructs with/without mitochondrial targeting sequence; CoQ10 measurement by HPLC PloS one Medium 24911838

Source papers

Stage 0 corpus · 57 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1993 Computer-assisted identification of a putative methyltransferase domain in NS5 protein of flaviviruses and lambda 2 protein of reovirus. The Journal of general virology 196 8385698
1996 Steady-state kinetics of the reduction of coenzyme Q analogs by complex I (NADH:ubiquinone oxidoreductase) in bovine heart mitochondria and submitochondrial particles. Biochemistry 134 8611577
1996 Enhanced sensitivity of ubiquinone-deficient mutants of Saccharomyces cerevisiae to products of autoxidized polyunsaturated fatty acids. Proceedings of the National Academy of Sciences of the United States of America 133 8755509
1997 Mitochondrial function is required for resistance to oxidative stress in the yeast Saccharomyces cerevisiae. FEBS letters 123 9237633
1999 Yeast and rat Coq3 and Escherichia coli UbiG polypeptides catalyze both O-methyltransferase steps in coenzyme Q biosynthesis. The Journal of biological chemistry 94 10419476
1996 Complementation of coq3 mutant yeast by mitochondrial targeting of the Escherichia coli UbiG polypeptide: evidence that UbiG catalyzes both O-methylation steps in ubiquinone biosynthesis. Biochemistry 92 8703953
2014 Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants. Biochimica et biophysica acta 85 24406904
2007 Saccharomyces cerevisiae Coq9 polypeptide is a subunit of the mitochondrial coenzyme Q biosynthetic complex. Archives of biochemistry and biophysics 81 17391640
2000 Genetic evidence for a multi-subunit complex in the O-methyltransferase steps of coenzyme Q biosynthesis. Biochimica et biophysica acta 70 10760477
2000 Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. The Journal of biological chemistry 69 10777520
2004 Genetic evidence for a multi-subunit complex in coenzyme Q biosynthesis in yeast and the role of the Coq1 hexaprenyl diphosphate synthase. The Journal of biological chemistry 66 15548532
1991 Ubiquinone biosynthesis in Saccharomyces cerevisiae. Isolation and sequence of COQ3, the 3,4-dihydroxy-5-hexaprenylbenzoate methyltransferase gene. The Journal of biological chemistry 65 1885593
2003 Yeast Coq5 C-methyltransferase is required for stability of other polypeptides involved in coenzyme Q biosynthesis. The Journal of biological chemistry 57 14701817
1990 Studies on the role of ubiquinone in the control of the mitochondrial respiratory chain. Free radical research communications 56 2354808
2014 Functional conservation of coenzyme Q biosynthetic genes among yeasts, plants, and humans. PloS one 55 24911838
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
1997 Sensitivity to treatment with polyunsaturated fatty acids is a general characteristic of the ubiquinone-deficient yeast coq mutants. Molecular aspects of medicine 51 9266513
1998 Genetic evidence for coenzyme Q requirement in plasma membrane electron transport. Journal of bioenergetics and biomembranes 48 9932649
1992 Coenzyme Q-pool function in glycerol-3-phosphate oxidation in hamster brown adipose tissue mitochondria. Journal of bioenergetics and biomembranes 48 1326518
2012 The submitochondrial distribution of ubiquinone affects respiration in long-lived Mclk1+/- mice. The Journal of cell biology 42 23045551
1978 Biophysical studies on agents affecting the state of membrane lipids: biochemical and pharmacological implications. Molecular and cellular biochemistry 39 154058
2013 The effects of statins on the mevalonic acid pathway in recombinant yeast strains expressing human HMG-CoA reductase. BMC biotechnology 35 24128347
2008 Comparison of a coq7 deletion mutant with other respiration-defective mutants in fission yeast. The FEBS journal 35 18808426
1994 Cloning of a rat cDNA encoding dihydroxypolyprenylbenzoate methyltransferase by functional complementation of a Saccharomyces cerevisiae mutant deficient in ubiquinone biosynthesis. Gene 35 8125303
2005 Inhibition of glycerophosphate-dependent H2O2 generation in brown fat mitochondria by idebenone. Biochemical and biophysical research communications 28 16300743
2016 A metabolomics-driven approach reveals metabolic responses and mechanisms in the rat heart following myocardial infarction. International journal of cardiology 26 27852445
2023 Mitochondrial matrix RTN4IP1/OPA10 is an oxidoreductase for coenzyme Q synthesis. Nature chemical biology 25 37884807
2005 C. elegans knockouts in ubiquinone biosynthesis genes result in different phenotypes during larval development. BioFactors (Oxford, England) 24 16873927
1994 Ubiquinone biosynthesis in eukaryotic cells: tissue distribution of mRNA encoding 3,4-dihydroxy-5-polyprenylbenzoate methyltransferase in the rat and mapping of the COQ3 gene to mouse chromosome 4. Archives of biochemistry and biophysics 23 8053692
2017 Differential expression and co-expression gene networks reveal candidate biomarkers of boar taint in non-castrated pigs. Scientific reports 22 28939879
2012 Restoring de novo coenzyme Q biosynthesis in Caenorhabditis elegans coq-3 mutants yields profound rescue compared to exogenous coenzyme Q supplementation. Gene 22 22735617
2021 Metabolic and Immunological Subtypes of Esophageal Cancer Reveal Potential Therapeutic Opportunities. Frontiers in cell and developmental biology 21 34307352
2016 AarF Domain Containing Kinase 3 (ADCK3) Mutant Cells Display Signs of Oxidative Stress, Defects in Mitochondrial Homeostasis and Lysosomal Accumulation. PloS one 21 26866375
2024 In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis. Nature catalysis 19 38425362
2020 Characterization of human mitochondrial PDSS and COQ proteins and their roles in maintaining coenzyme Q10 levels and each other's stability. Biochimica et biophysica acta. Bioenergetics 19 32194061
2019 Identification of an iron-responsive subtype in two children diagnosed with relapsing-remitting multiple sclerosis using whole exome sequencing. Molecular genetics and metabolism reports 16 30963028
2015 Structural and biochemical studies reveal UbiG/Coq3 as a class of novel membrane-binding proteins. The Biochemical journal 16 26251450
2021 Proteomic Response of Rat Pituitary Under Chronic Mild Stress Reveals Insights Into Vulnerability and Resistance to Anxiety or Depression. Frontiers in genetics 15 34603401
2021 Identification and Characterization of Non-Coding RNAs in Thymoma. Medical science monitor : international medical journal of experimental and clinical research 14 34219124
1998 Mitochondrial respiratory mutants in yeast inhibit glycogen accumulation by blocking activation of glycogen synthase. The Journal of biological chemistry 12 9813042
1993 Cardiostimulatory action of coenzyme Q homologues on cultured myocardial cells and their biochemical mechanisms. The Clinical investigator 12 8241709
2022 Candidate Modifier Genes for the Penetrance of Leber's Hereditary Optic Neuropathy. International journal of molecular sciences 11 36233195
2022 Exploring the common gene signatures and pathogeneses of obesity with Alzheimer's disease via transcriptome data. Frontiers in endocrinology 10 36568118
2018 Intracellular reduction of coenzyme Q homologues with a short isoprenoid side chain induces apoptosis of HeLa cells. Journal of biochemistry 9 29319808
2022 Predicting and Understanding the Pathology of Single Nucleotide Variants in Human COQ Genes. Antioxidants (Basel, Switzerland) 6 36552517
2002 Analysis of respiratory mutants reveals new aspects of the control of glycogen accumulation by the cyclin-dependent protein kinase Pho85p. FEBS letters 6 11943203
1995 A 29.425 kb segment on the left arm of yeast chromosome XV contains more than twice as many unknown as known open reading frames. Yeast (Chichester, England) 6 8533473
2022 Clinical course of a Japanese patient with developmental delay linked to a small 6q16.1 deletion. Human genome variation 5 35581197
2024 Association between coenzyme Q 10-related genetic polymorphisms and statin-associated myotoxicity in Korean stroke patients. Frontiers in pharmacology 4 38774208
2022 Levels of Coenzyme Q10 and Several COQ Proteins in Human Astrocytoma Tissues Are Inversely Correlated with Malignancy. Biomolecules 4 35204836
2016 Autism and intellectual disability in a patient with two microdeletions in 6q16: a contiguous gene deletion syndrome? Molecular cytogenetics 4 27980676
2025 Mitochondrial-ER Contact Sites and Tethers Influence the Biosynthesis and Function of Coenzyme Q. Contact (Thousand Oaks (Ventura County, Calif.)) 2 39906518
2026 Construction of a competitive endogenous RNA regulatory network for intramuscular fat content in Laiwu pig based on whole transcriptome analysis. Animal genetics 1 41581928
2024 Alterations in coenzyme Q10 status in a cybrid line harboring the 3243A>G mutation of mitochondrial DNA is associated with abnormal mitochondrial bioenergetics and dysregulated mitochondrial biogenesis. Biochimica et biophysica acta. Bioenergetics 1 38960080
2026 GOT2-mediated suppression of CoQ10 biosynthesis drives ferroptosis with divergent effects in lung adenocarcinoma and atherosclerosis. Communications biology 0 42215715
2025 Modelling the human coenzyme Q deficiency in Drosophila melanogaster. Free radical biology & medicine 0 39864756
2025 Proteomic Comparison between Hyphae and Spores Reveals Pathogenicity of Mucor Irregularis. Journal of proteome research 0 40692258

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