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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >polycystin-2, the protein mutated in autosomal dominant polycystic kidney disease (ADPKD), is a Ca~2+ -permeable nonselective cation channel
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polycystin-2, the protein mutated in autosomal dominant polycystic kidney disease (ADPKD), is a Ca~2+ -permeable nonselective cation channel

机译：polycystin-2是常染色体显性多囊肾疾病（ADPKD）中突变的蛋白，是一种Ca〜2 +渗透性非选择性阳离子通道

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摘要

Defects in polycystin-2, a ubiquitous transmembrane glycoprotein of unknown function. is a major cause of autosomal dominant polycystic kidney disease (ADPKD), whose manifestation entails the development of fluid-filled cysts in target organs. Here, we demonstrate that polycystin-2 is present in term human syncy- tiotrophoblast, where it behaves as a nonselective cation channel. Lipid bilayer reconstitution of polycystin-2-positive human syncy- tiotrophoblast apical membranes displayed a nonselective cation channel with multiple subconductance states, and a high perm- selectivity to Ca~2+. This channel was inhibited by anti-polycystin-2 antibody, Ca~2+, La~3+, Gd~3+, and the diuretic amiloride. Channel function by polycystin-2 was confirmed by patch-clamping exper- iments of polycystin-2 heterologously infected Sf9 insect cells. Further, purified insect cell-derived recombinant polycystin-2 and in wt translated human polycystin-2 had similar ion channel activity. The polycystin-2 channel may be associated with fluid accumulation and/or ion transport regulation in target epithelia. including placenta. Dysregulation of this channel provides a mech- anism for the onset and progression of ADPKD.
机译：未知功能的普遍存在的跨膜糖蛋白polycystin-2中的缺陷。是常染色体显性遗传性多囊肾疾病（ADPKD）的主要原因，其表现需要在靶器官中形成充满液体的囊肿。在这里，我们证明了多囊藻蛋白2存在于人类合体滋养层细胞中，它表现为非选择性阳离子通道。多囊藻蛋白2阳性的人类合体滋养层细胞顶端膜的脂双层重建显示出具有多个亚导电状态的非选择性阳离子通道，并且对Ca〜2 +具有高选择性。此通道被抗多囊藻蛋白2抗体，Ca〜2 +，La〜3 +，Gd〜3 +和利尿剂阿米洛利抑制。多囊藻蛋白2异源感染的Sf9昆虫细胞的膜片钳实验证实了多囊藻蛋白2的通道功能。此外，纯化的昆虫细胞来源的重组多囊藻蛋白-2和wt翻译的人多囊藻蛋白-2具有相似的离子通道活性。 polycystin-2通道可能与靶标上皮细胞中的液体蓄积和/或离子迁移调控有关。包括胎盘。该通道的失调为ADPKD的发作和发展提供了机制。

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