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已知85%～90%的常染色体显性遗传性多囊肾病是由pkd1突变引起的,因此pkd1编码的多囊蛋白-1的功能受到研究者的关注。多囊蛋白-1是一个具有长的细胞外氨基末端,11个跨膜区,短的细胞内羧基末端的跨膜蛋白。近年来发现多囊蛋白-1与多条信号转导通路有关。文章主要介绍多囊蛋白-1相关的PI3-K/Akt/mTOR、Wnt/β-catenin和JAK-STAT等信号转导通路、各信号通路之间的联系及其在常染色体显性遗传性多囊肾病致病中的作用。
It is known that 85% to 90% of autosomal dominant polycystic kidney disease is caused by the mutation of pkd1, so the function of pkd1-encoded polycystin-1 has drawn the attention of researchers. Polycystin-1 is a transmembrane protein with a long extracellular amino-terminal, 11 transmembrane domain, short intracellular carboxy-terminal. In recent years, polycystin-1 was found to be associated with multiple signal transduction pathways. This article mainly introduces the signal transduction pathways of PI3-K / Akt / mTOR, Wnt / β-catenin and JAK-STAT related to polycystin-1 and the relationship between the signal pathways and their autosomal dominant Cystic kidney disease in the role.