CD105/Endoglin is an auxiliary receptor for the TGF-β receptor complex, functioning in related signaling pathways. CD105/Endoglin is a transmembrane protein that exists as a disulfide-linked homodimer. It is mainly expressed in vascular and connective tissues and in endothelial and stromal cells. Upregulated CD105/endoglin expression has been reported during wound healing and tumor vascularization, and in inflammatory tissues and developing embryos. Mutations in CD105/endoglin have been found to be a causal factor of hereditary hemorrhagic telangiectasia (HHT), a disease characterized by malformation of vascular structure. The importance of this protein for normal and tumor vascular function makes it a good marker for endothelial cell proliferation as well as a potential therapeutic target in cancer.