TABLE OF CONTENTS Chapter 1: General Introduction 9 Chapter 2: The multi-faceted extracellular matrix: unlocking its secrets for understanding the perpetuation of lung fibrosis 17 Chapter 3: Abnormal collagen structure resulting from lack of contribution of collagen type XIV in lungs of patients with idiopathic pulmonary fibrosis 57 Chapter 4: Innovative 3D models for understanding mechanisms underlying lung diseases: powerful tools for translational research 71 Chapter 5: Dysregulated cross talk between alveolar epithelial cells and stromal cells in IPF reduces epithelial regenerative capacity 111 Chapter 6: 3D lung models – 3D extracellular matrix models 127 Chapter 7: Current possibilities and future opportunities with threedimensional lung ECM-derived hydrogels 165 Chapter 8: An in vitro model of fibrosis using crosslinked native extracellular matrix-derived hydrogels to modulate biomechanics without changing composition 183 Chapter 9: Fibroblast remodeling of extracellular matrix is directed by the fibrotic nature of the three-dimensional microenvironment 217 Chapter 10: General discussion and future perspectives 289 Appendices: English summary 306 Nederlandse samenvatting 309 Acknowledgements 313 Curriculum vitae 323 List of publications 324
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