Sibon group

• • All Publications: mepa page or overview (pdf file)

  • Selected Publications

   

  1. Yi Yu , Marianne van der Zwaag , Jouke Jan Wedman , Hjalmar Permentier , Niels Plomp , Xiu Jia , Bart Kanon , Ellie Eggens-Meijer , Girbe Buist , Hermie Harmsen , Jan Kok , Joana Falcao Salles , Bregje Wertheim , Susan J Hayflick , Erick Strauss , Nicola A Grzeschik , Hein Schepers , Ody C M Sibon. Coenzyme A precursors flow from mother to zygote and from microbiome to host.. Molecular Cell 2022 Jul 21;82(14):2650-2665.e12. doi: 10.1016/j.molcel.2022.05.006. (pdf)
  2. Anita I. E. Faber, Marianne van der Zwaag, Hein Schepers, Ellie Eggens-Meijer, Bart Kanon, Carmen IJsebaart, Jeroen Kuipers, Ben N.G. Giepmans, Raimundo Freire, Nicola A. Grzeschik, Catherine Rabouille, Ody C.M. Sibon. Vps13 is required for timely removal of nurse cell corpses. Development 2020. 147: dev191759 doi: 10.1242/dev.191759 (pdf)
  3. Suh Young Jeong, Penelope Hogarth, ….. , Ody C M Sibon, & Susan J 4’-Phosphopantetheine corrects CoA, iron and dopamine metabolic defects in mammalian models of PKAN. EMBO MOL MED 2019 Dec;11(12):e10489 (pdf)
  4. Roald Lambrechts, Yu Yi, Hein Schepers, Marianne van der Zwaag, Kaija Autio, Marina A. Tijssen, Susan Hayflick, Nicola Grzeschik, Ody Sibon. Linkage of CoA and pyruvate dehydrogenase reveals a pathway affected in neurodegenerative diseases. EMBO MOL MED 2019 Dec;11(12):e10488 . (pdf)  Artikel 59 and 59 are published back-to-back in the December issue 2019, the manuscripts were also honoured with the frontcover of this issue
  5. 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 OC. Human VPS13A is associated with multiple organelles and influences mitochondrial morphology and lipid droplet motility. eLife 2019 Feb 11;8. pii: e43561. (pdf)
  6. Iuso A, …., Sibon OCM & Anikster Y (2018). Mutations in PPCS, Encoding Phosphopantothenoylcysteine Synthetase, Cause Autosomal-Recessive Dilated Cardiomyopathy. Am J Hum Genet 2018 Jun; 102 (6): 1018-1030. (pdf) 
  7. Ody C.M. Sibon & Erick Strauss. Coenzyme A: make it or (re) uptake it. Nature Reviews in Molecular Cell Biology (2016) 10:605-606 (pdf) 
  8. 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. Extracellular 4’-Phosphopantetheine is a source for intracellular Coenzyme A synthesis. Nature Chemical Biology 2015 Oct;11(10):784-792. (pdf) Accompanied by a New and Views item
  9. Den Hoed M,….Sibon OCM*, et al. Heart rate-associated loci and their effects on cardiac conduction and rhythm disorders. (Nature Genetics 2013 Jun;45(6):621-31. (pdf)  *All included functional Drosophila melanogaster experiments of this GWAS study were chaired by Sibon OCM. 
  10. Katarzyna Siudeja K, Xu L, Srinivasan B, Rana A, Jong JJ,Jackowski S, Hayflick S and Sibon OCM. Impaired Coenzyme A metabolism affects histone and tubulin acetylation in a Drosophila and human cell model of the neurodegenerative disorder PKAN. EMBO MOL MED 2011 12:755-66 . (pdf) 
  11. Rana A, Seinen E, Siudeja K, Muntendam R, Srinivasan B, van der Want J, Hayflick S, Reijngoud D, Kayser O, Sibon OCM. Pantethine rescues a Drosophila model for Pantothenate Kinase-Associated Neurodegeneration. Proc Natl Acad Sci U S A. 2010 107:6988-93. (pdf)

The general consensus  that cells obtain their Coenzyme A via a de novo biosynthesis pathway starting with the uptake of vitamin B5 was recently challenged by work of the Sibon group, demonstrating, that cells and organisms can take up Coenzyme A from external sources. The mechanism behind this newly discovered manner of obtaining Coenzyme A is one of the topics of the Sibon lab. The presence of this alternative route of intracellular Coenzyme A is of high importance for subjects suffering from inborn genetic errors of impaired Coenzyme A de novo biosynthesis, resulting in severe neurodegenerative and cardiac  diseases. Fundamental basic research findings are translated into novel therapies for Coenzyme A-linked diseases.

Selected ongoing projects/grants:

2019   ZonMW from Bench to bedside: A phase-II study of a Food-For-Special-  Medical-Purposes (FSMP) for Pantothenate-Kinase-Associated-Neurodegeneration  (PKAN) enabling a cost- and time-effective approach to treat the disease

2019   NWO-ALW open aanvraag: Identification of the microbiome species and their kinases required for an inter-organismal Coenzyme A synthesis pathway

2017   NWO-ALW open aanvraag: Identification of external sources of Coenzyme A for cells and organisms.

Selected previous projects/grants:

2011    VICI NWO grant: Title: Coenzyme A; back in the spotlight

2011    EU KP7 Health: Title: TIRCON: Treating iron-related childhood onset neurodegeneration.

2002    VIDI NWO grant: Title: Characterization of Drosophila mutants sensitive or insensitive to genotoxic stress (P.I.)

Dissertations supervised by Ody C.M. Sibon ((co-) promotor or assessor):

2019

Lambrechts, R. A. (2019). Childhood-onset movement disorders: mechanistic and therapeutic insights from Drosophila melanogaster. [Groningen]: Rijksuniversiteit Groningen. https://doi.org/10.33612/diss.101316004

Faber, A. I. E. (2019). VPS13A: shining light on its localization and function. [Groningen]: Rijksuniversiteit Groningen.

2018

Pinto, F. (2018).  Unraveling VPS13A pathways: from Drosophila to human. [Groningen]; University of Groningen

Monteiro Morgado, L. (2018). Computational Methods for High-Throughput Small RNA Analysis in Plants [Groningen]: University of Groningen

de Boer, P. (2018). Correlative microscopy reveals abnormalities in type 1 diabetes[Groningen]: Rijksuniversiteit Groningen

Li, Y. (2018). The identification of cell non-autonomous roles of astrocytes in neurodegeneration [Groningen]: University of Groningen

Yeshaw, W. M. (2018). VPS13A is a multitasking protein at the crossroads between organelle communication and protein homeostasis [Groningen]: University of Groningen

2017

Vonk, J. (2017). Characterization of a Drosophila model for Chorea‐Acanthocytosis[Groningen]: University of Groningen

Dunham, J. T-N. (2017). Translational multiple sclerosis research in primates: Mind the gap [Groningen]: Rijksuniversiteit Groningen

2016

Alvarenga Fernandes Sin, O. (2016). Aggregation-promoting factors in neurodegenerative diseases: Insights from a C. elegans model [Groningen]: University of Groningen

Baratashvili, M. B. (2016). Rescue strategies in Drosophila models of neurodegenerative diseases [Groningen]: Rijksuniversiteit Groningen

Smeets, C. J. L. M. (2016). The molecular neuropathology of spinocerebellar ataxia type 23 [Groningen]: Rijksuniversiteit Groningen

Laturney, M. E. (2016). The second sex: Functions and mechanisms of sperm manipulation in female Drosophila melanogaster [Groningen]: Rijksuniversiteit Groningen

2015

Srinivasan, B. (2015). Rerouting ‘coenzyme A’ biosynthesis [Groningen]: University of Groningen

2012

Siudeja, K. A. (2012). Coenzyme A as a central player in cellular and tissue homeostasis [S.n.]

2011

Seinen, E. (2011). Small regulatory RNAs: identification, classification and utilizations.n.

Zijlstra, M. P. (2011). The role of heat shock proteins in polyQ disorders Groningen: s.n.

2010

Rana, A. (2010). The role of impaired de novo Coenzyme A biosynthesis in pantothenate kinase-associated neurodegeneration: insight from a Drosophila modelGroningen: s.n.

2009

Vos, M. (2009). Small heat shock proteins: Implications for neurodegeneration & longevity s.n.

Student projects:

• Understanding the uptake mechanism of Coenzyme A and its precursors from external sources.
• Exploring the possibilities of patented compounds to treat Pantothenate Kinase-Associated Neurodegeneration.
• Developing a Google-Drosophila tool to study the ultrastructure of the Drosophila brain

PhD students:
Hilda de Vries
Ria Hut
Floris Bosveld
Xia Yi
Erwin Seinen
Katarzina Siudeja
Anil Rana
Balaji Srinivasan
Marianne de Villiers
Madina Baratashvili

Interesting websites linked to the Sibon lab

• www.tircon.eu
• www.nanotomy.org