ArticleMolecular cell2019
An Isoprene Lipid-Binding Protein Promotes Eukaryotic Coenzyme Q Biosynthesis.
Article in Molecular cell, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 44 citations in OpenAlex.
- Uridine-sensitized screening identifies demethoxy-coenzyme Q and NUDT5 as regulators of nucleotide synthesis.Nature metabolism · 2025Article
- An ETFDH-driven metabolon supports OXPHOS efficiency in skeletal muscle by regulating coenzyme Q homeostasis.Nature metabolism · 2024Article
- In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis.Nature catalysis · 2024Article
- Biallelic variants in COQ7 cause distal hereditary motor neuropathy with upper motor neuron signs.Brain : a journal of neurology · 2023Article
- Mutant SPART causes defects in mitochondrial protein import and bioenergetics reversed by Coenzyme Q.Open biology · 2023Article
- Characterization ofMetabolites · 2023Article
- Coenzyme Q biochemistry and biosynthesis.Trends in biochemical sciences · 2023Review
- CoQAntioxidants (Basel, Switzerland) · 2023Article
- Predicting and Understanding the Pathology of Single Nucleotide Variants in HumanAntioxidants (Basel, Switzerland) · 2022Article
- Structure and functionality of a multimeric human COQ7:COQ9 complex.Molecular cell · 2022Article
- Manganese-driven CoQ deficiency.Nature communications · 2022Article
- How plants synthesize coenzyme Q.Plant communications · 2022Review
- Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes.Journal of medical genetics · 2022Article
- Recent advances in the metabolic pathways and microbial production of coenzyme Q.World journal of microbiology & biotechnology · 2022Review
- A unique flavoenzyme operates in ubiquinone biosynthesis in photosynthesis-related eukaryotes.Science advances · 2021Article
- Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings.Antioxidants (Basel, Switzerland) · 2021Review
- Cellular Models for Primary CoQ Deficiency Pathogenesis Study.International journal of molecular sciences · 2021Review
- UbiB proteins regulate cellular CoQ distribution in Saccharomyces cerevisiae.Nature communications · 2021Article
- CLoNe: automated clustering based on local density neighborhoods for application to biomolecular structural ensembles.Bioinformatics (Oxford, England) · 2021Article
- Mitochondrial Lipid Signaling and Adaptive Thermogenesis.Metabolites · 2021Review
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Authors and funding
18 authors at 4 institutions in 2 countries.
Funding
Abstract
The biosynthesis of coenzyme Q presents a paradigm for how cells surmount hydrophobic barriers in lipid biology. In eukaryotes, CoQ precursors-among nature's most hydrophobic molecules-must somehow be presented to a series of enzymes peripherally associated with the mitochondrial inner membrane. Here, we reveal that this process relies on custom lipid-binding properties of COQ9. We show that COQ9 repurposes the bacterial TetR fold to bind aromatic isoprenes with high specificity, including CoQ intermediates that likely reside entirely within the bilayer. We reveal a process by which COQ9 associates with cardiolipin-rich membranes and warps the membrane surface to access this cargo. Finally, we identify a molecular interface between COQ9 and the hydroxylase COQ7, motivating a model whereby COQ9 presents intermediates directly to CoQ enzymes. Overall, our results provide a mechanism for how a lipid-binding protein might access, select, and deliver specific cargo from a membrane to promote biosynthesis.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.