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Hereditary hemorrhagic telangiectasia (HHT) type 2 is an autosomal dominant disease in which one allele of the <i>ACVRL1</i> gene is mutated. Patients exhibit disturbances in TGF-beta/BMP-dependent angiogenesis and, clinically, often present with severe nosebleeds as well as a reduced quality of life. The aim of our study was to use CRISPR/Cas9 to knockout <i>ACVRL1</i> in normal induced pluripotent stem cells (iPSCs) and evaluate the effects on TGF-beta- and BMP-related gene expression as well as angiogenesis. The CRISPR/Cas9 knockout of the <i>ACVRL1</i> gene was carried out in previously characterized wild-type (<i>ACVRL1^wt/wt</i>) iPSCs. An HHT type 2 iPS cell line was generated via a single-allele knockout (<i>ACVRL1^wt/mut</i>) in wild-type (<i>ACVRL1^wt/wt</i>) iPSCs, resulting in a heterozygous 17 bp frameshift deletion in the <i>ACVRL1</i> gene [NG_009549.1:g.13707_13723del; NM_000020.3:c.1137_1153del]. After the generation of embryoid bodies (EBs), endothelial differentiation was induced via adding 4 ng/mL BMP4, 2% B27, and 10 ng/mL VEGF. Endothelial differentiation was monitored via immunocytochemistry. An analysis of 151 TGF-beta/BMP-related genes was performed via RT-qPCR through the use of mRNA derived from single iPS cell cultures as well as endothelial cells derived from EBs after endothelial differentiation. Differential TGF-beta/BMP gene expression was observed between <i>ACVRL1^wt/wt</i> and <i>ACVRL1^wt/mut</i> iPSCs as well as endothelial cells. EBs derived from CRISPR/Cas9-designed <i>ACVRL1</i> mutant HHT type 2 iPSCs, together with their isogenic wild-type iPSC counterparts, can serve as valuable resources for HHT type 2 in vitro studies.