Platelet-derived growth factor (PDGF) acts as a potent mitogen, chemoattractant and survival factor for mesenchymal cells. In addition to its importance in mammalian development, PDGF plays a critical role in physiological repair mechanisms and in the pathogenesis of various proliferative diseases. The biological effects of PDGF are initiated via two related receptor tyrosine kinases, termed α and β PDGF receptors. Recent observations provide increasing evidence for distinct roles of the two PDGF receptor subtypes in both embryogenesis and disease formation. Moreover, characterization of the signal relay mechanisms indicates, that the α and β PDGF receptors are not identical in their ability to bind intracellular effector molecules. Furthermore, the two PDGF receptors initiate overlapping, yet distinct signal transduction pathways. These differences may account for some of the variabilities in biological responses resulting from activation of these two receptors.