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Retinopathy of prematurity (ROP) is a kind of vasoproliferative disorders, which leads to vision loss even blindness in premature neonates. Major pathogenesis in ROP is vascular occlusion and retinal neovascularization precipitated by ischemia and hypoxia. Proliferative retinopathy is asscioated with several signaling pathways, such as Wnt signaling pathway, CCN1/cysteine-rich 61(CCN1/Cry61) in pathological neovascularization, JAK/STAT (Janus kinase/signal transducer and activator of transcription) in intravitreous neovascularization, Apelin/APJ (Apelin/angiotensin type 1 receptor related protein) in pathological retinal angiogenesis. Deep understanding of the pathways is conducive to treat proliferative retinopathy by targeting pathologic neovessels.