In vitro and In vivo models for studying endothelial cell development and hereditary hemorrhagic telangiectasia

Chapter 2 investigated the generation of knockin mESC line in order to study the biallelic distribution of Nanog expression at the protein level. Chapter 3 investigated the generation of human ECs from hESCs using a spin-EB differentiation approach. Chapter 4 described the rst derivation of three human iPS cell lines in the Netherlands: two from healthy individuals and one from a patient with HHT. Chapter 5 investigated the use of hiPSC-EC as a model for studying HHT1 in vitro.Chapter 6 investigated the role of Gja5 that encodes for the gap junction protein Cx40 in the development of AVMs in the HHT2 mouse model.