New perspective of ClC-Kb/2 Cl- channel physiology in the distal renal tubule.
New perspective of ClC-Kb/2 Cl- channel physiology in the distal renal tubule.
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DOI：
10.1152/ajprenal.00577.2015
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发表时间：
2016-05
期刊：
影响因子：
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通讯作者：
Oleg L. Zaika;Viktor N Tomilin;M. Mamenko;Vivek Bhalla;O. Pochynyuk
中科院分区：
文献类型：
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作者：
Oleg L. Zaika;Viktor N Tomilin;M. Mamenko;Vivek Bhalla;O. Pochynyuk
由于其被鉴定为Bartter综合征3型的潜在分子原因，因此提出ClC-Kb（啮齿动物中的ClC-K2，此后将其称为ClC-Kb/2）通过控制从皮质厚升支到外髓集合管的远端肾小管段中的基底外侧Cl（-）出口在全身电解质平衡和血压调节中发挥重要作用。相当多的实验和临床工作致力于鉴定和表征致病突变以及通过其辅因子barttin控制该通道。然而，我们才刚刚开始解开ClC-Kb/2在不同肾小管节段中的作用，并揭示其表达和功能的调节因子，例如，胰岛素和IGF-1。在这篇综述中，我们讨论了最近在这方面的实验证据，并强调未探索的问题，关键是要了解ClC-Kb/2在肾脏的生理。
Since its identification as the underlying molecular cause of Bartter's syndrome type 3, ClC-Kb (ClC-K2 in rodents, henceforth it will be referred as ClC-Kb/2) is proposed to play an important role in systemic electrolyte balance and blood pressure regulation by controlling basolateral Cl(-) exit in the distal renal tubular segments from the cortical thick ascending limb to the outer medullary collecting duct. Considerable experimental and clinical effort has been devoted to the identification and characterization of disease-causing mutations as well as control of the channel by its cofactor, barttin. However, we have only begun to unravel the role of ClC-Kb/2 in different tubular segments and to reveal the regulators of its expression and function, e.g., insulin and IGF-1. In this review we discuss recent experimental evidence in this regard and highlight unexplored questions critical to understanding ClC-Kb/2 physiology in the kidney.