碳酸氢钠协同转运蛋白：结构、功能与调节

The sodium bicarbonate cotransporter: structure, function, and regulation.

作者信息

Bernardo Angelito A, Bernardo Christian M, Espiritu Doris Joy, Arruda Jose A L

机构信息

Department of Medicine, Section of Nephrology, University of Illinois at Chicago, 820 S. Wood Street, Chicago, IL 60612, USA.

出版信息

Semin Nephrol. 2006 Sep;26(5):352-60. doi: 10.1016/j.semnephrol.2006.07.008.

DOI:10.1016/j.semnephrol.2006.07.008

PMID:17071329

Abstract

The role of the Na(+)-coupled HCO(3)(-) transporter (NBC) family is indispensable in acid-base homeostasis. Almost all tissues express a member of the NBC family. NBC has been studied extensively in the kidney and plays a role in proximal tubule HCO(3)(-) reabsorption. Although the exact function of this transporter family on other tissues is not very clear, the ubiquitous expression of NBC family suggests a role in cell pH regulation. Altered NBC activity caused by mutations of the gene responsible for NBC protein expression results in pathophysiologic conditions. Mutations of NBC resulting in important clinical disorders have been reported extensively on one member of the NBC family, the kidney NBC (NBC1). These mutations have led to several structural studies to understand the mechanism of the abnormal NBC1 activity.

摘要

钠耦联碳酸氢根转运体（NBC）家族在酸碱平衡中发挥着不可或缺的作用。几乎所有组织都表达NBC家族的一个成员。NBC在肾脏中已得到广泛研究，并在近端小管碳酸氢根重吸收中发挥作用。尽管该转运体家族在其他组织中的具体功能尚不完全清楚，但NBC家族的广泛表达提示其在细胞pH调节中发挥作用。负责NBC蛋白表达的基因突变导致的NBC活性改变会引发病理生理状况。关于NBC家族的一个成员——肾脏NBC（NBC1），已广泛报道了导致重要临床疾病的NBC突变。这些突变促使开展了多项结构研究，以了解异常NBC1活性的机制。