Pozzobon Tommaso, Goldoni Giacomo, Viola Antonella, Molon Barbara
Department of Biomedical Sciences, University of Padua, Padua, Italy; Venetian Institute of Molecular Medicine (VIMM), Padova, Italy.
Venetian Institute of Molecular Medicine (VIMM), Padova, Italy.
Immunol Lett. 2016 Sep;177:6-15. doi: 10.1016/j.imlet.2016.06.006. Epub 2016 Jun 27.
Chemokines and chemokine receptors regulate multiple processes such morphogenesis, angiogenesis and immune responses. Among the chemokine receptors, CXCR4 stands out for its pleiotropic roles as well as for its involvement in several pathological conditions, including immune diseases, viral infections and cancer. For these reasons, CXCR4 represents a crucial target in drug development. In this review, we discuss of CXCR4 receptor properties and signaling in health and diseases, focusing on the WHIM syndrome, an inherited immunodeficiency caused by mutations of the CXCR4 gene.
趋化因子和趋化因子受体调节多种过程,如形态发生、血管生成和免疫反应。在趋化因子受体中,CXCR4因其多效性作用以及参与包括免疫疾病、病毒感染和癌症在内的多种病理状况而脱颖而出。基于这些原因,CXCR4是药物开发中的关键靶点。在本综述中,我们讨论CXCR4受体在健康和疾病中的特性及信号传导,重点关注由CXCR4基因突变引起的遗传性免疫缺陷疾病——疣状肢端角化不良综合征(WHIM综合征)。