The hydroxylation of lysine residues by lysyl hydroxylase is essential for the formation of stable collagen fibrils. This post-translational modification allows collagen molecules to form cross-links, which are necessary for the tensile strength and structural integrity of tissues. Deficiencies in lysyl hydroxylase activity can lead to disorders such as Ehlers-Danlos syndrome and osteogenesis imperfecta, which are characterized by fragile connective tissues.
