Principal Investigators
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
  • Research Profile
  • Selected Publications
  • Ody Sibon is Full Professor at the University Medical Center Groningen and Principal Investigator at the European Research Institute for the Biology of Ageing, where her research focuses on the fundamental mechanisms underlying age-related neurodegeneration, with a particular emphasis on Coenzyme A (CoA) metabolism. Her group investigates how disruptions in CoA biosynthesis contribute to rare neurological and cardiac diseases, including Pantothenate Kinase-Associated Neurodegeneration (PKAN), using a multidisciplinary approach that combines yeast, Drosophila melanogaster, mammalian cell systems, and mouse models. A central theme of her work is understanding how cells acquire CoA, including alternative uptake pathways beyond the classical vitamin B5-dependent biosynthesis route, as well as the interaction between host metabolism and the microbiome. Her research has direct translational relevance, exemplified by a clinical trial she coordinated and initiated in 2021 evaluating safety and tolerability of 4’-phosphopantetheine (4’-PPT) supplementation in PKAN patients. Sibon obtained her MSc and PhD from Utrecht University and completed her postdoctoral training at Stony Brook University with support from a fellowship from the Netherlands Organization for Scientific Research, which also awarded her prestigious Vidi (2002) and Vici (2011) grants. She is actively involved in European research consortia focused on rare diseases and the development of novel therapeutic strategies.

    • 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.
    • 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.
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