I am a cell biologist at University Medical Centre Groningen (UMCG), Groningen, the Netherlands. I specialise in chaperone-dependent protein quality control (PQC), i.e. the way by which cells handle proteins on their way to functionality and how they take care of proteins that are wrongly folded or prone to intracellular clumping (aggregation). Because protein aggregates hallmark polyQ diseases like Huntington’s disease (HD) and Spinocerebellar Ataxias (SCAs), I have studied the role of chaperones in preventing toxic protein aggregation in such diseases, in particular.
I completed my study and training as a biologist at the University of Groningen. I initially worked in the field of Radiation Oncology at UMCG to study radiation in combination with hyperthermia (i.e. temperature elevation that also induces aggregation). Around 2000, I moved to the Department of Medical Cell Biology (also UMCG) and gradually switched from studying acute protein stress (hyperthermia) to chronic protein stress (polyQ diseases). Since 2010, I have been in close contact with HD patients and their family members and they have joined me for very impressive teaching classes for a variety of student groups before I actually start my cell biology teaching.
In my PhD work (Groningen University), I focused on the biological effect of hyperthermia. This causes protein aggregation in cells and make these cell more sensitive to chemo and radiotherapy. After a short stay in the US, I learned how heat shock proteins (HSPs) as so-called molecular chaperones could prevent or repair aggregates and this knowledge I exploit until today for my research after aggregates formed in polyQ diseases. In 2010, we discovered a set of chaperones that very effectively prevent the toxic aggregation of polyQ proteins, and this is what we have been trying to exploit since.
I collaborate with many PQC and polyQ diseases researchers from all over the world, including many clinicians. With a few Dutch HD researchers, we started the Dutch Huntington Disease Research Network (DHDRN) and actively supported the Campaign Team Huntington in their efforts to raise money for HD-research
Within the CureQ consortium, I coordinate WP4 aimed at the discovery of novel modulators disease onset and progression and with my colleague Mark Hipp (UMCG) we will run a project (PhD still to be appointed) on early reporters of the collapse of the protein homeostasis in cells as biomarker for disease onset.