The Sibon’s group focus is to understand Coenzyme A metabolism in health and disease. The textbook classic, Coenzyme A is a central metabolic cofactor, known for over 60 years. In addition to its essential role in metabolism it also plays a role in posttranslational modifications of proteins, signal transduction, ageing, epigenetics, neurodegeneration and more.
Moleculaire en celbiologie
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):
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.
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
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
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
Srinivasan, B. (2015). Rerouting ‘coenzyme A’ biosynthesis [Groningen]: University of Groningen
Siudeja, K. A. (2012). Coenzyme A as a central player in cellular and tissue homeostasis [S.n.]
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.
Vos, M. (2009). Small heat shock proteins: Implications for neurodegeneration & longevity s.n.
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Hilda de Vries
Marianne de Villiers
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