NBCe1 as a model carrier for understanding the structure-function properties of Na⁺ -coupled SLC4 transporters in health and disease.
NBCe1 as a model carrier for understanding the structure-function properties of Na⁺ -coupled SLC4 transporters in health and disease.
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DOI：
10.1007/s00424-014-1448-8
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发表时间：
2014-08
影响因子：
4.5
通讯作者：
Kurtz, Ira
中科院分区：
文献类型：
--
作者：
Kurtz, Ira
SLC 4转运蛋白是通常介导碳酸氢盐（碳酸盐）的偶联转运并共享氨基酸序列同源性的膜蛋白。这些蛋白质除了它们的电荷传输化学计量、膜靶向、底物亲和力、发育表达、调控基序和蛋白质-蛋白质相互作用之外，它们是否也传输Na+和/或Cl−。这些差异部分解释了这样一个事实，即在功能上，SLC 4转运蛋白在哺乳动物中具有各种生理作用，包括跨上皮碳酸氢盐转运、细胞内pH调节、Na+和/或Cl-转运以及可能的水转运。Bicycle转运不是SLC 4家族所独有的，因为结构上不相关的SLC 26家族至少有三种蛋白质介导Cl−-HCO 3 −交换。本审查的重点是第一个钠依赖性SLC 4转运蛋白，确定其结构已被最广泛的研究：产电Na+-碱协同转运NBCe 1。NBCe 1突变引起近端肾小管酸中毒（pRTA），伴有神经系统和眼科肾外表现。最近的研究已经表征了转运蛋白的重要结构-功能特性，以及它们如何由于导致pRTA的突变而受到干扰。越来越明显的是，NBCe 1的结构与SLC 4 Cl−-HCO 3 −交换剂AE 1在几个关键特征上不同，后者的结构性质已经得到了充分的研究。在这篇综述中，NBCe 1的结构-功能特性和调节将被强调，其在健康和疾病中的作用将被详细审查。
SLC4 transporters are membrane proteins that in general mediate the coupled transport of bicarbonate (carbonate) and share amino acid sequence homology. These proteins differ as to whether they also transport Na+ and/or Cl−, in addition to their charge transport stoichiometry, membrane targeting, substrate affinities, developmental expression, regulatory motifs, and protein-protein interactions. These differences account in part for the fact that functionally, SLC4 transporters have various physiological roles in mammals including transepithelial bicarbonate transport, intracellular pH regulation, transport of Na+ and/or Cl−, and possibly water. Bicarbonate transport is not unique to the SLC4 family since the structurally unrelated SLC26 family has at least three proteins that mediate Cl−-HCO3− exchange. The present review focuses on the first of the sodium-dependent SLC4 transporters that was identified whose structure has been most extensively studied: the electrogenic Na+-base cotransporter NBCe1. Mutations in NBCe1 cause proximal renal tubular acidosis (pRTA) with neurologic and ophthalmologic extrarenal manifestations. Recent studies have characterized important structure-function properties of the transporter and how they are perturbed as a result of mutations that cause pRTA. It has become increasingly apparent that the structure of NBCe1 differs in several key features from the SLC4 Cl−-HCO3− exchanger AE1 whose structural properties have been well-studied. In this review, the structure-function properties and regulation of NBCe1 will be highlighted and its role in health and disease will be reviewed in detail.
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影响因子：
14.5
作者：
Bosley, Thomas M.;Salih, Mustafa A.;Abu-Amero, Khaled K.
通讯作者：
Abu-Amero, Khaled K.
DOI：
10.1152/ajprenal.00131.2002
发表时间：
2003-06-01
影响因子：
4.2
作者：
Choi, I;Hu, LH;Boron, WF
通讯作者：
Boron, WF
DOI：
10.1152/ajprenal.00015.2008
发表时间：
2008-09-01
影响因子：
4.2
作者：
Azimov, Rustam;Abuladze, Natalia;Kurtz, Ira
通讯作者：
Kurtz, Ira
影响因子：
4.8
作者：
Chang, Min-Hwang;DiPiero, Jennifer;Romero, Michael F.
通讯作者：
Romero, Michael F.
同时用 NBCn1 替换假定的人 NBCe1-A TM6 和 TM12 对非洲爪蟾卵母细胞表面丰度的影响
影响因子：
2.4
作者：
Chen, Li-Ming;Qin, Xue;Boron, Walter F.
通讯作者：
Boron, Walter F.