Ion channels in renal disease.

Ion channels in renal disease.
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DOI:
10.1021/cr3001077
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发表时间:
2012-12-12
期刊:
影响因子:
62.1
通讯作者:
Ehrlich, Barbara E.
Ehrlich, Barbara E.
中科院分区:
化学1区
文献类型:
--
作者:
Kuo, Ivana Y.;Ehrlich, Barbara E.

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肾脏细胞含有许多专门的离子通道和转运蛋白,它们协同作用,通过吸收或分泌离子到尿液中来调节体积和离子浓度。肾脏中参与离子过滤和浓缩的每个区域都表达特定的离子通道子集。这些离子通道共同确保适当的电解质稳态。然而,许多遗传和基因突变使这些通道功能失调或丧失功能。这些离子通道中的一种或多种的突变与多种症状相关,包括蛋白尿、肾功能进行性丧失和肾性高血压。肾功能逐渐丧失,最终导致终末期肾病,通常通过透析或移植来治疗。无论从患病率还是经济负担来看,终末期肾病都是一个日益严重的健康问题。本次综述的范围是首先提供肾脏和功能的总体概述,然后具体讨论突变时导致肾脏疾病的离子通道。
The cells of the kidney contain many specialized ion channels and transporters, which act in concert to regulate volume and ionic concentration by absorption or secretion of ions into the urine. Each region of the kidney involved in filtration and concentration of ions expresses a particular subset of ion channels. Together, these ion channels ensure appropriate electrolyte homeostasis. However, a number of hereditary and genetic mutations render these channels dys-or nonfunctional. Mutations to one or more of these ion channels are associated with a variety of symptoms including proteinuria, progressive loss of renal function, and renal hypertension. The progressive loss of renal function, culminating in end-stage renal disease, is typically treated by dialysis or transplantation. End-stage renal disease is an increasing health problem, both in terms of prevalence and economic burden. The scope of this review is to first provide a general overview of the kidney and function, and then specifically address the ion channels that, when mutated, lead to kidney disease.
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