Lysyl oxidase (LOX) is a copper-dependent amine oxidase that plays a pivotal role in the assembly of the extracellular matrix (ECM) by catalyzing the oxidative deamination of lysine residues in collagen and elastin, leading to the formation of aldehydes that participate in the cross-linking of these structural proteins. This process is essential for tissue integrity and repair. Beyond its enzymatic function, LOX also engages in a variety of signaling pathways, contributing to cell fate, differentiation, and communication during development, tissue maintenance, and repair. LOX activities have been implicated in a range of disorders, including those affecting the vasculature, heart, lungs, skin, placenta, diaphragm, kidneys, and pelvic floor, as well as in conditions like glioblastoma, diabetic neovascularization, osteogenic differentiation, and tumor progression and metastasis. In the context of inflammation, LOX is involved in reducing pluripotent mesenchymal cell pools, which are relevant to the pathology of diseases such as diabetes, osteoporosis, and rheumatoid arthritis. LOX also interacts with signaling mediators like EGFR, PDGF, VEGF, and TGF-β, influencing processes such as angiogenesis, macrophage infiltration, and glioma cell survival. Its multifaceted functions make LOX a significant player in both normal physiological processes and the pathogenesis of various diseases.