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In this translational project, you will apply recently developed zebrafish Lyso-IP/-Tag lines to interrogate intra-lysosomal proteomes, lipidomes and metabolomes in cln3-deficient models. The goals are to (i) uncover lysosomal pathways perturbed by CLN3 loss using LC-MS-based proteomics and metabolomics of enriched lysosomes from larvae and adult tissues (eye, brain), and (ii) test how different energy sources (lipids, glucose, amino acids) influence disease biomarkers (e.g., GPDs) and lysosomal morphology in vivo. You will work closely with Dr Peter van Hasselt (paediatrician for metabolic diseases), core facilities (Proteomics, Lipidomics, Metabolomics), and collaborators at the Hubrecht Institute.
Key responsibilities
You will join the Research Section of the Department of Genetics (UMC Utrecht), embedded in a dynamic translational setting with close ties to the Wilhelmina Children’s Hospital (WKZ) and Center for Molecular Medicine (CMM). The project is part of the UMC Utrecht Child Health priority program and benefits from collaborations with the Hubrecht Institute, AUMC Metabolomics, and clinical partners. Our location at the Utrecht Science Park enables extensive interactions and joint projects with neighboring institutes.

