ArticleMolecular cell2016
Cerebellar Ataxia and Coenzyme Q Deficiency through Loss of Unorthodox Kinase Activity.
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Article in Molecular cell, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 68 papers, 1 of them a synthesis that pooled it.
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68citing papers in PubMed, 1 pooled it
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Who cites it
68 citing papers in PubMed, 1 synthesis or guideline pooled it, 122 citations in OpenAlex.
- The efficacy of coenzyme QJournal of cellular and molecular medicine · 2022Pooled it
- Recurrent posterior stroke-like lesions heralding COQ8A-related coenzyme Q10 deficiency: a potentially treatable ataxia mistaken for stroke: a case report.Journal of neurology · 2026Article
- Serum Peptidomic Analysis in a Comparative Study of the Efficacy of Benjakul Remedy Versus Loratadine in Allergic Rhinitis Patients.International journal of molecular sciences · 2026Article
- COQ8 chaperones coenzyme Q lipid intermediates through ATP-driven structural gating.Science advances · 2026Article
- Spatial mapping of CoQ10 repletion by BPM31510 in a genetic mouse model (Coq4Journal of lipid research · 2026Article
- Cooperative Architecture of Mitochondrial Proteome Homeostasis.medRxiv : the preprint server for health sciences · 2026Article
- Non-canonical role of natural quinones in mitochondrial nucleoid organization for maintaining respiration and protecting cardiac function.Journal of biochemistry · 2026Article
- Cerebellar deep brain stimulation rescues Purkinje cell mitochondrial density in a genetic mouse model of cerebellar ataxia.Brain research bulletin · 2026Article
- Article
- Autosomal Recessive Cerebellar Ataxias: Translating Genes to Therapies.Annals of neurology · 2025Review
- The ADCK Kinase Family: Key Regulators of Bioenergetics and Mitochondrial Function and Their Implications in Human Cancers.International journal of molecular sciences · 2025Review
- Arabidopsis conditional photosynthesis mutants abc1k1 and var2 accumulate partially processed thylakoid preproteins and are defective in chloroplast biogenesis.Communications biology · 2025Article
- Prenatal and progressive coenzyme QJournal of cachexia, sarcopenia and muscle · 2024Article
- Bi-allelic variants in COQ8B, a gene involved in the biosynthesis of coenzyme Q10, lead to non-syndromic retinitis pigmentosa.American journal of human genetics · 2024Article
- Understanding coenzyme Q.Physiological reviews · 2024Review
- Mitochondrial Dysfunction due to Novel COQ8A Variation with Poor Response to CoQ10 Treatment: A Comprehensive Study and Review of Literatures.Cerebellum (London, England) · 2024Review
- Toll-like receptor 4 deficiency in Purkinje neurons drives cerebellar ataxia by impairing the BK channel-mediated after-hyperpolarization and cytosolic calcium homeostasis.Cell death & disease · 2024Article
- Identification of Potent ADCK3 Inhibitors through Structure-Based Virtual Screening.Journal of chemical information and modeling · 2024Article
- Primary Coenzyme Q10 Deficiency-Related Ataxias.Journal of clinical medicine · 2024Review
- Review
8 more citing papers are in PubMed but not listed here.
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Authors and funding
38 authors at 11 institutions in 3 countries.
Jonathan A StefelyMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Floriana LicitraDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France.
Leila LaredjDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France.
Andrew G ReidenbachMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Zachary A KemmererMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Anais GrangerayUniversité de Strasbourg, 67081 Strasbourg, France; Institut des Neurosciences Cellulaires et Intégratives, CNRS UPR 3212, 67084 Strasbourg, France.
Tiphaine Jaeg-EhretDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France.
Catherine E MinogueDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Arne UlbrichDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Paul D HutchinsDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Emily M WilkersonDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Zheng RuanInstitute of Bioinformatics, University of Georgia, Athens, GA 30602, USA; Department of Biochemistry & Molecular Biology, University of Georgia, Athens, GA 30602, USA.
Deniz AydinInstitute of Bioengineering, School of Life Sciences, École Polytechnique Fédérale de Lausanne, 1015 Lausanne, Switzerland; Swiss Institute of Bioinformatics, 1015 Lausanne, Switzerland.
Alexander S HebertGenome Center of Wisconsin, University of Wisconsin-Madison, Madison, WI 53706, USA.
Xiao GuoMorgridge Institute for Research, Madison, WI 53715, USA; Department of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Elyse C FreibergerDepartment of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Laurence ReutenauerDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France.
Adam JochemMorgridge Institute for Research, Madison, WI 53715, USA.
Maya ChergovaDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France.
Isabel E JohnsonMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Danielle C LohmanMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Matthew J P RushDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Nicholas W KwiecienDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Pankaj K SinghDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France.
Anna I SchlagowskiFédération de Medicine Translationnelle de Strasbourg, EA3072, Faculté de Médicine et Faculté des Sciences du Sport, Université de Strasbourg, 67084 Strasbourg, France.
Brendan J FloydMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Ulrika ForsmanUniversity Grenoble Alpes, LCBM, UMR 5249, 38000 Grenoble, France.
Pavel J SindelarUniversity Grenoble Alpes, LCBM, UMR 5249, 38000 Grenoble, France; Laboratoire de Chimie des Processus Biologiques, CNRS UMR 8229, Collège de France, 75252 Paris, France.
Michael S WestphallGenome Center of Wisconsin, University of Wisconsin-Madison, Madison, WI 53706, USA.
Fabien PierrelUniversity Grenoble Alpes, LCBM, UMR 5249, 38000 Grenoble, France; TIMC-IMAG, CNRS UMR 5525, UFR de Médecine, University Joseph Fourier, 38706 La Tronche, France.
Joffrey ZollFédération de Medicine Translationnelle de Strasbourg, EA3072, Faculté de Médicine et Faculté des Sciences du Sport, Université de Strasbourg, 67084 Strasbourg, France.
Matteo Dal PeraroInstitute of Bioengineering, School of Life Sciences, École Polytechnique Fédérale de Lausanne, 1015 Lausanne, Switzerland; Swiss Institute of Bioinformatics, 1015 Lausanne, Switzerland.
Natarajan KannanInstitute of Bioinformatics, University of Georgia, Athens, GA 30602, USA; Department of Biochemistry & Molecular Biology, University of Georgia, Athens, GA 30602, USA.
Craig A BingmanDepartment of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Joshua J CoonDepartment of Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA; Genome Center of Wisconsin, University of Wisconsin-Madison, Madison, WI 53706, USA; Department of Biomolecular Chemistry, University of Wisconsin-Madison, Madison, WI 53706, USA.
Philippe IsopeUniversité de Strasbourg, 67081 Strasbourg, France; Institut des Neurosciences Cellulaires et Intégratives, CNRS UPR 3212, 67084 Strasbourg, France.
Hélène PuccioDépartement de Médecine Translationnelle et Neurogénétique, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U596, CNRS UMR 7104, 67400 Illkirch, France; Université de Strasbourg, 67081 Strasbourg, France; Chaire de Génétique Humaine, Collège de France, 67404 Illkirch, France. Electronic address: hpuccio@igbmc.fr.
David J PagliariniMorgridge Institute for Research, Madison, WI 53715, USA; Department of Biochemistry, University of Wisconsin-Madison, Madison, WI 53706, USA. Electronic address: dpagliarini@morgridge.org.
University of Wisconsin–Madison · USMorgridge Institute for Research · USUniversité de Strasbourg · FRCentre National de la Recherche Scientifique · FRCollège de France · FRInstitut de génétique et de biologie moléculaire et cellulaire · FRUniversity of Georgia · USÉcole Polytechnique Fédérale de Lausanne · CHLaboratoire de Chimie et Biologie des Métaux · FRSIB Swiss Institute of Bioinformatics · CHUniversité Grenoble Alpes · FR
Funding
Research Training for Computation and Informatics in Biology and MedicineT15LM007359 · NLM · UNIVERSITY OF WISCONSIN-MADISON · PI Mark W. Craven, Colin Noel Dewey · 2002 to 2026
$22.6MBIOTECHNOLOGY TRAINING PROGRAMT32GM008349 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI FOX, BRIAN G · 1989 to 2019
$22.5MINTEGRATED TRAINING FOR PHYSICIAN-SCIENTISTST32GM008692 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI BURKARD, MARK E · 1998 to 2020
$10.3MChemistry-Biology Interface Training ProgramT32GM008505 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI BLACKWELL, HELEN E. · 1993 to 2023
$9.8MTRAINING IN NUTRITIONT32DK007665 · NIDDK · UNIVERSITY OF WISCONSIN-MADISON · PI GUY E GROBLEWSKI, Chi- Liang Eric Yen · 1993 to 2026
$9.2MStructure, Function and Regulation of the ProteomeR35GM118110 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI COON, JOSHUA J · 2016 to 2025
$9.1MRESEARCH TRAINING IN HEMATOLOGYT32HL007899 · NHLBI · UNIVERSITY OF WISCONSIN-MADISON · PI Jane Ellen Churpek · 1998 to 2026
$8.2MPartnership for High-Throughput Enabled Biology of the Mitochondrial ProteomeU01GM094622 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI MARKLEY, JOHN LUTE · 2010 to 2014
$7.8MRegulation of Mitochondrial Metabolism by Post-Translational ModificationsR01DK098672 · NIDDK · WASHINGTON UNIVERSITY · PI David J Pagliarini · 2013 to 2026
$4.8MThe Transmembrane Protein CenterU54GM094584 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI FOX, BRIAN G · 2010 to 2014
$4.6MEnzyme Discovery for Natural Product BiosynthesisU01GM098248 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI PHILLIPS, GEORGE NEAL · 2011 to 2015
$2.8MEstablishing the role of the atypical kinase ADCK3 in mitochondrial metabolismR01GM112057 · NIGMS · UNIVERSITY OF WISCONSIN-MADISON · PI PAGLIARINI, DAVID J · 2014 to 2017
$1.3MNCI NIH HHS Y01 CO1020-11NHLBI NIH HHS T32 HL007899NIA NIH HHS F30 AG043282NIDDK NIH HHS R01 DK098672NIDDK NIH HHS T32 DK007665NIGMS NIH HHS R01 GM112057NIGMS NIH HHS R01 GM115591NIGMS NIH HHS R35 GM118110NIGMS NIH HHS T32 GM008349NIGMS NIH HHS T32 GM008505NIGMS NIH HHS T32 GM008692NIGMS NIH HHS U01 GM094622NIGMS NIH HHS U01 GM098248NIGMS NIH HHS U54 GM094584NIGMS NIH HHS Y01 GM1104-11NLM NIH HHS T15 LM007359
Abstract
The UbiB protein kinase-like (PKL) family is widespread, comprising one-quarter of microbial PKLs and five human homologs, yet its biochemical activities remain obscure. COQ8A (ADCK3) is a mammalian UbiB protein associated with ubiquinone (CoQ) biosynthesis and an ataxia (ARCA2) through unclear means. We show that mice lacking COQ8A develop a slowly progressive cerebellar ataxia linked to Purkinje cell dysfunction and mild exercise intolerance, recapitulating ARCA2. Interspecies biochemical analyses show that COQ8A and yeast Coq8p specifically stabilize a CoQ biosynthesis complex through unorthodox PKL functions. Although COQ8 was predicted to be a protein kinase, we demonstrate that it lacks canonical protein kinase activity in trans. Instead, COQ8 has ATPase activity and interacts with lipid CoQ intermediates, functions that are likely conserved across all domains of life. Collectively, our results lend insight into the molecular activities of the ancient UbiB family and elucidate the biochemical underpinnings of a human disease.
Indexed as
Behavior, AnimalAnimalsCerebellar AtaxiaCerebellumChlorocebus aethiopsCOS CellsDisease Models, AnimalExercise ToleranceFemaleGenetic Predisposition to DiseaseHEK293 CellsHumansLipid MetabolismMaleMaze LearningMice, Inbred C57BLCOQ8A protein, humanCOQ8 protein, S cerevisiaeMitochondrial ProteinsSaccharomyces cerevisiae ProteinsUbiquinoneubiquinone 8
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