Proper synthesis and assembly of extracellular matrix components is essential to provide cells and tissues with their biological and biomechanical properties. Biosynthesis of collagen, the most important component of the extracellular matrix, requires the commitment of a set of enzymatic components, including prolyl and lysyl hydroxylases and lysyl oxidases, responsible for a number of post-translational modifications, many of them unique for collagen. The action of these collagen remodeling enzymes is essential to provide the collagen molecule with its capacity to deposit on the extracellular matrix and form supramolecular structures like bones, teeth or blood vessels, and, therefore, they exert an important influence in homeostasis and disease. By the establishment of different cellular and animal models, in our group, we investigate the molecular mechanisms regulating the biological activity of collagen remodeling enzymes, as well as their pathophysiological relevance in the development of fibrosis and cardiovascular diseases.