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[Physiology and pathophysiology of Na(+)/HCO3(-) cotransporter NBCe1.] [Article in Chinese]

LIU Ying, LU QunWei, CHEN LiMing

Department of Biophysics and Molecular Physiology； Department of Genetics and Developmental Biology； Key Laboratory of Molecular Biophysics of Ministry of Education, School of Life Science and Technology, Huazhong University of Science and Technology, Wuhan 430074, China.

Abstract

Na(+)/HCO3(-) cotransporter NBCe1 is an electrogenic member of the solute carrier 4 (SLC4) family and plays important roles in intracellular pH regulation as well as transepithelial HCO3(-) movement. The physiological and pathological significance of NBCe1 has been well established by genetic studies with humans as well as knock-out study with mouse. NBCe1 is expressed in diverse tissues in mammals. The transporter plays an essential role in the maintenance of acid-base homeostasis in our body, being responsible for more ~80% of HCO3(-) reabsorption in the proximal renal tubule. In humans, a number of SLC4A4 mutations have been associated with proximal renal tubule acidosis that is often accompanied with short stature, ocular abnormalities (including cataract, glaucoma, and band keratopathy), migraine, and/or defects in dental enamel development. In the present article, we review the molecular physiology, the structure/function relationship, the mechanisms underlying the functional regulation of NBCe1, as well as the physiological and pathological roles of the transporter.

Key words: bicarbonate transporter; acid-base balance; renal tubule; migraine; metabolic acidosis; SLC4A4

Received: 2012-03-11　　Accepted: 2012-05-30

Corresponding author: 陈历明　　E-mail: liming.chen@mail.hust.edu.cn

Citing This Article：

LIU Ying, LU QunWei, CHEN LiMing. [Physiology and pathophysiology of Na(+)/HCO3(-) cotransporter NBCe1.] [Article in Chinese] . Acta Physiol Sin 2012; 64 (6): 729-740 (in Chinese with English abstract).