Principal Investigators

Ody Sibon

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.

    • Hayflick SJ & Sibon OCM (2026) Neurodegeneration with brain iron accumulation – from gene discovery to therapeutic strategies Nature Reviews in Neurology (accepted 2nd Oct 2026)
    • 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. (2025) Opt1 imports CoA precursors as glutathione mixed disulfides. Journal of Biological Chemistry.
    • Böttcher J, Sibon OCM, El Aidy S. (2025) Coenzyme A metabolism: a key driver of gut microbiota dynamics and metabolic profiles. FEMS Microbiology Reviews.
    • 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. (2022) Coenzyme A precursors flow from mother to zygote and from microbiome to host. Molecular Cell.
    • 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. (2020) Vps13 is required for timely removal of nurse cell corpses. Development.
    • Roald Lambrechts, Yu Yi, Hein Schepers, Marianne van der Zwaag, Kaija Autio, Marina A. Tijssen, Susan Hayflick, Nicola Grzeschik, Ody Sibon. (2019) CoA-dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases. EMBO Molecular Medicine.
    • Suh Young Jeong, Penelope Hogarth, Andrew Placzek, Allison M Gregory, Rachel Fox, Dolly Zhen, Jeffrey Hamada, Marianne van der Zwaag, Roald Lambrechts, Haihong Jin, Aaron Nilsen, Jared Cobb, Thao Pham, Nora Gray, Martina Ralle, Megan Duffy, Leila Schwanemann, Puneet Rai, Alison Freed, Katrina Wakeman, Randy Woltjer, Ody C.M. Sibon, & Susan J Hayflick. (2019). 4’-Phosphopantetheine corrects CoA, iron and dopamine metabolic defects in mammalian models of PKAN. EMBO Molecular Medicine.
    • Yeshaw WM, van der Zwaag M, Pinto F, Lahaye LL, Faber AI, Gómez-Sánchez R, Dolga AM, Poland C, Monaco AP, van IJzendoorn S, Grzeschik NA, Velayos-Baeza A, Sibon OCM. (2019) Human VPS13A is associated with multiple organelles and influences mitochondrial morphology and lipid droplet motility. eLife
    • Ody C.M. Sibon & Erick Strauss (2016) Coenzyme A: to make it or uptake it? Nature reviews.
    • B. Srinivasan, M. Baratashvili, M. Van der Zwaag, B. Kanon, C. Colombelli, R.A. Lambrechts, O. Schaap, E.A.A. Nollen, A. Podgoršek, G. Kosec, H. Petković, S. Hayflick, V. Tiranti, D.J. Reijngoud, N.A. Grzeschik, Ody C.M. Sibon (2015) Extracellular 4’-Phosphopantetheine is a source for intracellular Coenzyme A synthesis. Nature Chemical Biology
    • Den Hoed M. et al , Sibon OCM, Milan DJ, Schnieder H, Samani NJ, Loos RJF. (2013) Identification of Heart rate-associated loci and their effects on cardiac conduction and rhythm disorders. Nature Genetics
    • Katarzyna Siudeja K, Xu L, Srinivasan B, Rana A, Jong JJ,Jackowski S, Hayflick S and Sibon OCM (2011) Impaired Coenzyme A metabolism affects histone and tubulin acetylation in a Drosophila and human cell model of the neurodegenerative disorder PKAN. EMBO Molecular Medicine
    • Rana A, Seinen E, Siudeja K, Muntendam R, Srinivasan B, van der Want J, Hayflick S, Reijngoud D, Kayser O, Sibon OCM. (2010) Pantethine rescues a Drosophila model for Pantothenate Kinase-Associated Neurodegeneration. The Proceedings of the National Academy of Sciences U S A.
    • Bosveld F, Rana A, van der Wouden P, Lemstra W, Ritsema M, Kampinga HH, Sibon OCM. (2008) De novo CoA biosynthesis is required to maintain DNA integrity during development of the Drosophila nervous system. Human Molecular Genetics
    • Sibon OCM. (2003) Centrosomes as DNA damage regulators. Nature Genetics.
    • Khodjakov A, Rieder CL, Sluder G, Cassels G, Sibon OCM, Wang CL. (2002) De novo formation of centrosomes in vertebrate cells arrested during S phase. Journal of Cell Biology.
    • Sibon OCM, Kelkar A, Lemstra W, Theurkauf WE. (2000) DNA-replication/DNA-damage-dependent centrosome inactivation in Drosophila embryos. Nature Cell Biology.
    • Sibon OCM, Laurencon A, Hawley R, Theurkauf WE. (1999) The Drosophila ATM homologue Mei-41 has an essential checkpoint function at the midblastula transition. Current Biology.
    • Sibon OCM, Stevenson VA, Theurkauf WE. (1997) DNA-replication checkpoint control at the Drosophila midblastula transition. Nature.
    • Luderus ME, van Steensel B, Chong L, Sibon OCM, Cremers FF, de Lange T.(1996) Structure, subnuclear distribution, and nuclear matrix association of the mammalian telomeric complex. Journal of Cell Biology.
    • Humbel BM, Sibon OCM, Stierhof YD, Schwarz H. (1995) Ultra-small gold particles and silver enhancement as a detection system in immunolabeling and in situ hybridization experiments. Journal of Histochemistry & Cytochemistry
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