钾转运体的生物生成:疾病相关突变的影响。

IF 6.2 2区 生物学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY Critical Reviews in Biochemistry and Molecular Biology Pub Date : 2024-06-01 Epub Date: 2024-07-01 DOI:10.1080/10409238.2024.2369986
Morgan Kok, Jeffrey L Brodsky
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引用次数: 0

摘要

由于各种离子转运体、通道和泵的作用,细胞内和细胞外钾的浓度受到严格调节,而这些离子转运体、通道和泵主要位于肾脏。然而,钾转运体和共转运体在所有器官和细胞类型中都发挥着重要作用。也许不足为奇的是,这些蛋白质的生物发生、功能和/或调节缺陷与一系列灾难性人类疾病有关,但迄今为止,治疗这些疾病的药物很少获得批准。在这篇综述中,我们将讨论一组钾-氯共转运体(KCCs)以及相关的钠-钾-氯共转运体(NKCCs)的结构、功能和活性。此外,还讨论了与四种 KCC 和两种 NKCC 相关的疾病。我们特别强调了这些复杂的膜蛋白如何在内质网中折叠和成熟,非原生形式的共转运体如何在细胞中被破坏,以及哪些细胞因素会监督它们的成熟和向细胞表面的转运。如果已知,我们还将概述如何调节每种共转运体的水平和活性。我们还进一步概述了该领域的未决问题和未来的研究方向。
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The biogenesis of potassium transporters: implications of disease-associated mutations.

The concentration of intracellular and extracellular potassium is tightly regulated due to the action of various ion transporters, channels, and pumps, which reside primarily in the kidney. Yet, potassium transporters and cotransporters play vital roles in all organs and cell types. Perhaps not surprisingly, defects in the biogenesis, function, and/or regulation of these proteins are linked to range of catastrophic human diseases, but to date, few drugs have been approved to treat these maladies. In this review, we discuss the structure, function, and activity of a group of potassium-chloride cotransporters, the KCCs, as well as the related sodium-potassium-chloride cotransporters, the NKCCs. Diseases associated with each of the four KCCs and two NKCCs are also discussed. Particular emphasis is placed on how these complex membrane proteins fold and mature in the endoplasmic reticulum, how non-native forms of the cotransporters are destroyed in the cell, and which cellular factors oversee their maturation and transport to the cell surface. When known, we also outline how the levels and activities of each cotransporter are regulated. Open questions in the field and avenues for future investigations are further outlined.

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来源期刊
CiteScore
14.90
自引率
0.00%
发文量
6
期刊介绍: As the discipline of biochemistry and molecular biology have greatly advanced in the last quarter century, significant contributions have been made towards the advancement of general medicine, genetics, immunology, developmental biology, and biophysics. Investigators in a wide range of disciplines increasingly require an appreciation of the significance of current biochemical and molecular biology advances while, members of the biochemical and molecular biology community itself seek concise information on advances in areas remote from their own specialties. Critical Reviews in Biochemistry and Molecular Biology believes that well-written review articles prove an effective device for the integration and meaningful comprehension of vast, often contradictory, literature. Review articles also provide an opportunity for creative scholarship by synthesizing known facts, fruitful hypotheses, and new concepts. Accordingly, Critical Reviews in Biochemistry and Molecular Biology publishes high-quality reviews that organize, evaluate, and present the current status of high-impact, current issues in the area of biochemistry and molecular biology. Topics are selected on the advice of an advisory board of outstanding scientists, who also suggest authors of special competence. The topics chosen are sufficiently broad to interest a wide audience of readers, yet focused enough to be within the competence of a single author. Authors are chosen based on their activity in the field and their proven ability to produce a well-written publication.
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