TABLE OF CONTENTS Chapter 1 General introduction 7 Chapter 2 Cortical interneuron development is affected in 4H leukodystrophy Brain, 2023, 146(7), 2846-2860 35 Chapter 3 Investigating neuron intrinsic defects in 4H and Globoid Leukodystrophy 79 Chapter 4 Towards a 3D spheroid system for modelling leukodystrophies 109 Chapter 5 Human pluripotent stem cell-derived microglia shape neuronal morphology and enhance network activity in vitro Adapted from: Journal of Neuroscience Methods, 2025, Volume 415, 110354 155 Chapter 6 POLR3 gene and protein expression dynamics in 4H leukodystrophy using iPSC-derived neuronal lineages Under revision; Stem Cell Research, 2025 189 Chapter 7 Discussion 213 Appendix Summary Dankwoord / Acknowledgements 231 236
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