红细胞水合作用的决定因素。
Determinants of erythrocyte hydration.
机构信息
Department of Genetics, Yale University School of Medicine, New Haven, CT, USA.
出版信息
Curr Opin Hematol. 2010 May;17(3):191-7. doi: 10.1097/MOH.0b013e32833800d0.
PURPOSE OF REVIEW
Maintenance of cellular water and solute homeostasis is critical for survival of the erythrocyte. Inherited or acquired disorders that perturb this homeostasis jeopardize the erythrocyte, leading to its premature destruction. This study reviews recent progress in our understanding the determinants of erythrocyte hydration and its related disorders.
RECENT FINDINGS
The molecular and genetic bases of primary disorders of erythrocyte hydration are poorly understood. Recent studies have implicated roles for the anion transporter, SLC4A1, and the Rh-associated glycoprotein, RhAG. The most common secondary disorder associated with perturbed hydration of the erythrocyte is sickle cell disease, in which dehydration contributes to disease pathology and clinical complications. Advances in understanding the mechanisms regulating erythrocyte solute and water content, particularly associated with KCl cotransport and Gardos channel activation, have revealed novel signaling mechanisms controlling erythrocyte hydration. These signaling pathways may provide innovative strategies to prevent erythrocyte dehydration in sickle cell disease.
SUMMARY
Clinical, translational and biologic studies all contribute to our knowledge of erythrocyte hydration. Understanding the mechanisms controlling erythrocyte water and solute homeostasis will serve as a paradigm for other cells and may reveal new therapeutic targets for disease prevention and treatment.
目的综述
维持细胞内水和溶质的平衡对红细胞的存活至关重要。遗传性或获得性的破坏这种平衡的疾病会危及红细胞，导致其过早破坏。本研究综述了近年来我们对红细胞水合作用及其相关疾病决定因素的理解的最新进展。
最近的发现
原发性红细胞水合紊乱的分子和遗传基础了解甚少。最近的研究表明阴离子转运蛋白 SLC4A1 和 Rh 相关糖蛋白 RhAG 起作用。与红细胞水合紊乱最相关的常见继发性疾病是镰状细胞病，其中脱水导致疾病病理和临床并发症。对调节红细胞溶质和含水量的机制的理解的进展，特别是与 KCl 共转运和 Gardos 通道激活相关的进展，揭示了控制红细胞水合的新信号机制。这些信号通路可能为预防镰状细胞病中的红细胞脱水提供创新策略。
总结
临床、转化和生物学研究都有助于我们了解红细胞水合作用。理解控制红细胞水和溶质平衡的机制将为其他细胞提供一个范例，并可能为疾病的预防和治疗揭示新的治疗靶点。
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