Groep Sibon

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