Renée Maas

Chapter 1 General Introduction and Thesis Outline 11
Chapter 2 Harnessing Developmental Cues for Cardiomyocyte Production 27
Chapter 3 Wnt Activation and Reduced Cell-Cell Contact Synergistically Induce Massive Expansion of Functional Human iPSC-Derived Cardiomyocytes 55
Chapter 4 Massive Expansion and Cryopreservation of Functional Human Induced Pluripotent Stem cell-Derived Cardiomyocytes 87
Chapter 5 Sarcomere Disassembly and Transfection Efficiency in Proliferating Human iPSC-Derived Cardiomyocytes 107
Chapter 6 Metabolic Maturation Increases Susceptibility to Hypoxia-Induced Damage in Human iPSC-Derived Cardiomyocytes 125
Chapter 7 Phospholamban p.Arg14del Cardiomyopathy: a Systematic Summary of the Pathophysiological Mechanisms 149
Chapter 8 Transcriptional Regulation Profiling Reveals PPARA-mediated Fatty Acid Oxidation as a Novel Therapeutic Target in Phospholamban R14del Cardiomyopathy 199
Chapter 9 Unfolded Protein Response as a Compensatory Mechanism and Potential Therapeutic Target in PLN R14del Cardiomyopathy 239
Chapter 10 Generation of Human Induced Pluripotent Stem Cell (hiPSC) Lines Derived from Six Patients Carrying the Pathogenic Phospholamban R14del (PLN-R14del) Variant and Two non-carrier Family Members 263
Chapter 11 Generation, High Throughput Screening, and Biob anking of Human iPSC-Derived Cardiac Spheroids 277
Chapter 12 Modeling and Rescue of PLN-R14del Cardiomyopathy Phenotype in Human Induced Pluripotent Stem Cell-Derived Cardiac Spheroids 297
Chapter 13 General Discussion - hiPSC-CMs Disease Modelling and Future Perspectives 347
Chapter 14 General Summary 383
Summary in Dutch 390
List of publications 395
Acknowledgements 401
About the author 409
UU PhD Portfolio 411

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