Erik de Vrieze PhD

Over Erik de Vrieze

It is my goal to develop tailor-made (genetic therapies) for patients with inherited (adult-onset) hearing loss, or combined hearing loss and vision loss (Usher syndrome). These disorders have a major impact on the individual’s ability to communicate with others, and can ultimately lead to social isolation. Currently, there is no cure for inherited hearing loss, only techniques to artificially amplify hearing such as hearing aids of cochlear implants. For the vision problems associated with Usher syndrome, there is no treatment at all.

A dominantly-inherited form of hearing loss and vestibular dysfunction named DFNA9 is one of the major causes of adult-onset hearing loss in the Netherlands and Belgium. In 2017, I was awarded a ZonMW Off-road grant to explore the ability of specifically blocking the synthesis of mutant allele-derived cytotoxic proteins as a potential treatment for dominantly-inherited disorders such as DFNA9. Around the same time, a group of Dutch DNFA9 patients established their patient organisation "De Negende Van, www.denegendevan.nl", which whom I work in close contact to establish novel treatments for their disorder. The DFNA9 foundation recently supported my research by granting my team, and my collaborators from Antwerp University, the funds to develop an elegant, humanised mouse model to study their disorder. Recently, I published the first insights into a potential genetic treatment for DFNA9 on BioRXiv.

My second major interest in Usher Syndrome, for which we are slowly but steadily taking steps towards a better understanding of the disorder, and a future treatment for the patients vision loss. Zebrafish take a central role in my research on Usher syndrome. Recently, I published a beautiful paper together colleagues from London in which zebrafish models for Usher syndrome gave a first look into how patients with Usher syndrome gradually loose their sight. Zebrafish were also at the basis of the antisense oligonucleotide-based treatment that is currently being developed by ProQR Therapeutics.

My past research contains a diverse spectrum of research topics that are joined by the exploit zebrafish as a unique model for the advancement of science. Topics include endocrinology, skeletal biology and (retinal) ciliopathies. The unique possibilities that zebrafish offer a model species in biomedical research is also one of the key topics in my course on disease models for Biomedical Science studies at the Radboud University Medical Center.