About our research group/lab
Our research
In our lab we study the molecular mechanisms of von Willebrand factor secretion in endothelial cells in order to understand how defects in these mechanism can lead to bleeding disorders.
Using innovative (patient-derived) cellular model systems such as endothelial colony forming cells (ECFCs; formerly known as blood outgrowth endothelial cells, BOECs) we are systematically investigating the mechanisms that control how secretory organelles such as Weibel-Palade bodies (WPBs) are formed and how they acquire the molecular machinery to undergo exocytosis.
Over the years we have made substantial contributions to the elucidation of the signaling networks and the role of membrane proteins, such as Rab GTPases, Rab effectors and SNARE proteins, in the formation and exocytosis of WPBs.
Together with our clinical partners within the Erasmus MC (Prof. dr. Frank Leebeek, Hematology and Prof. dr. Marjon Cnossen, Pediatric Hematology) and beyond we use patient-derived ECFCs to study the pathogenic mechanisms of bleeding disorders such as von Willebrand disease (VWD). We also use this model system as a pre-clinical platform to evaluate gene correction and gene therapy strategies for von Willebrand disease (VWD), with the ultimate aim to develop personalized therapies for VWD patients.
Collaborations
Internal collaboration
- Prof. dr. Frank Leebeek (Hematology)
- Prof. dr. Marjon Cnossen (Pedriatric Hematology)
External collaborations:
NWO-TTW program on personalized medicine for von Willebrand disease:
- Prof. dr. Jan Voorberg (Sanquin Research and Landsteiner Laboratory, Amsterdam)
- Prof. dr. Jeroen Eikenboom (Leiden University Medical Center, Leiden)
NWO-NWA SYMPHONY consortium "Orchestrating personalised treatment for patients with bleeding disorders"
- Prof. dr. Geert van den Bogaart (Rijksuniversiteit Groningen)
- Prof. dr. Tom Carter (St. George's University London, United Kingdom)
Funding & Grants
Our team
- Ruben Bierings, principal investigator
- Petra Burgisser, research technician
- Iris van Moort, postdoc
- Marije Kat, PhD student (Sanquin)
- Sebastiaan Laan, PhD student (LUMC)
