Research Groups

Coenzyme A metabolism in health and disease

Ody Sibon’s research focuses on understanding the fundamental mechanisms underlying age-related neurodegeneration, with a particular emphasis on Coenzyme A (CoA) metabolism. Within the Sibon laboratory, research is directed at uncovering how disruptions in CoA biosynthesis contribute to rare neurological and cardiac diseases, including Pantothenate Kinase-Associated Neurodegeneration (PKAN). The lab applies a multidisciplinary approach, combining yeast, Drosophila melanogaster, mammalian cell systems, and mouse models to investigate how cells acquire, regulate, and utilize CoA.

A central aim of Ody Sibon’s work is to identify and characterize alternative pathways through which cells obtain CoA, challenging the traditional view that CoA is exclusively synthesized via vitamin B5-dependent pathways. The Sibon lab further explores how these mechanisms can be leveraged to develop novel therapeutic strategies. In addition, the group studies the interplay between host metabolism and the microbiome, with a particular focus on the exchange of CoA precursors between microbial communities and their host.

Overall, Ody Sibon’s research integrates fundamental and translational approaches, with the aim of advancing mechanistic insight and developing innovative treatments for CoA-related neurodegenerative and cardiac diseases .

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  • Publications
  • Ody Sibon Visit
    Position

    Group leader of the Coenzyme A metabolism in health and disease

    Research fields

    Coenzyme A (CoA) metabolism, Neuroacanthocytosis, Pantothenate Kinase-Associated Neurodegeneration (PKAN), Neurodegeneration & Brain iron metabolism

    • Hayflick SJ, Jeong SY, Sibon OCM. PKAN pathogenesis and treatment. Mol Genet Metab. 2022 Nov;137(3):283-291. doi: 10.1016/j.ymgme.2022.09.011. Epub 2022 Oct 5. PMID: 36240582; PMCID: PMC9970616.
    • Kiyuna LA, Odendaal C, Singh M, Gerding A, Langelaar-Makkinje M, van der Zwaag M, Drachman A, Cetkovská V, Kioen GLF, Martines AMF, Huijkman NCA, Schepers H, van de Sluis B, Reijngoud DJ, Sibon OCM, Harms AC, Hankemeier T, Bakker BM. Energetic stress in combination with impaired fatty acid oxidation induces sequestration of CoA and adaptation of CoA metabolism. FEBS J. 2026 Feb 7. doi: 10.1111/febs.70442. Epub ahead of print. PMID: 41652904.
    • Grzeschik NA, Lambrechts RA, van der Zwaag M, Eggens-Meijer E, Sibon OCM, Gorter JA. Dysregulation of Glial Gap Junction Protein Innexin2 Mediates Seizures and Disrupted Sleep in a Drosophila Model for North Sea Progressive Myoclonus Epilepsy. J Neurosci Res. 2025 Nov;103(11):e70087. doi: 10.1002/jnr.70087. PMID: 41208742; PMCID: PMC12598673.
    • Zhang F, Dorn T, Gnutti B, Anikster Y, Kuebler S, Ahrens-Nicklas R, Gosselin R, Rahman S, Durst R, Zanuttigh E, Güra MA, Poch CM, Meier AB, Laugwitz KL, Schüller HJ, Messias AC, Sibon OC, Finazzi D, Rippert A, Li D, Truxal K, Nandi D, Lampert BC, Yeo M, Gardham A, Nissan B, Horowitz Cederboim S, Moretti A, Iuso A. Pantethine ameliorates dilated cardiomyopathy features in PPCS deficiency disorder in patients and cell line models. Commun Med (Lond). 2025 Jul 31;5(1):323. doi: 10.1038/s43856-025-01017-z. PMID: 40745475; PMCID: PMC12313872.
    • Wedman JJ, de Vries L, van Lingen B, van der Zwaag M, Gómez-Sánchez R, Hardenberg R, Huibers W, Permentier H, Strauss E, Chang M, Reggiori F, de Kroon AI, Sibon OCM, Schepers H. Opt1 imports CoA precursors as glutathione mixed disulfides. J Biol Chem. 2025 Sep;301(9):110503. doi: 10.1016/j.jbc.2025.110503. Epub 2025 Jul 21. PMID: 40701247; PMCID: PMC12406275.
    • Böttcher J, Sibon OCM, El Aidy S. Coenzyme A metabolism: a key driver of gut microbiota dynamics and metabolic profiles. FEMS Microbiol Rev. 2025 Jan 14;49:fuaf051. doi: 10.1093/femsre/fuaf051. PMID: 41074819; PMCID: PMC12570885.
    • Polet SS, de Koning TJ, Lambrechts RA, Tijssen MAJ, Sibon OCM, Gorter JA. Conventional and novel anti-seizure medications reveal a particular role for GABAA in a North Sea progressive myoclonus Epilepsy Drosophila model. Epilepsy Res. 2024 Jul;203:107380. doi: 10.1016/j.eplepsyres.2024.107380. Epub 2024 May 14. PMID: 38781737.
    • Yu Y, van der Zwaag M, Wedman JJ, Permentier H, Plomp N, Jia X, Kanon B, Eggens-Meijer E, Buist G, Harmsen H, Kok J, Salles JF, Wertheim B, Hayflick SJ, Strauss E, Grzeschik NA, Schepers H, Sibon OCM. Coenzyme A precursors flow from mother to zygote and from microbiome to host. Mol Cell. 2022 Jul 21;82(14):2650-2665.e12. doi: 10.1016/j.molcel.2022.05.006. Epub 2022 Jun 3. PMID: 35662397.
    • Mostert KJ, Sharma N, van der Zwaag M, Staats R, Koekemoer L, Anand R, Sibon OCM, Strauss E. The Coenzyme A Level Modulator Hopantenate (HoPan) Inhibits Phosphopantotenoylcysteine Synthetase Activity. ACS Chem Biol. 2021 Nov 19;16(11):2401-2414. doi: 10.1021/acschembio.1c00535. Epub 2021 Sep 28. PMID: 34582681; PMCID: PMC8726015.
    • Yu Y, Moretti IF, Grzeschik NA, Sibon OCM, Schepers H. Coenzyme A levels influence protein acetylation, CoAlation and 4'-phosphopantetheinylation: Expanding the impact of a metabolic nexus molecule. Biochim Biophys Acta Mol Cell Res. 2021 Apr;1868(4):118965. doi: 10.1016/j.bbamcr.2021.118965. Epub 2021 Jan 13. PMID: 33450307.
    • Faber AIE, van der Zwaag M, Schepers H, Eggens-Meijer E, Kanon B, IJsebaart C, Kuipers J, Giepmans BNG, Freire R, Grzeschik NA, Rabouille C, Sibon OCM. Vps13 is required for timely removal of nurse cell corpses. Development. 2020 Oct 29;147(20):dev191759. doi: 10.1242/dev.191759. PMID: 32994170.
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