Veizis I Elias, Cotton Calvin U
Department of Pediatrics and Physiology and Biophysics, Rainbow Center for Childhood PKD, Case Western Reserve University, Cleveland, OH 44106, USA.
Pediatr Nephrol. 2007 Jun;22(6):770-7. doi: 10.1007/s00467-006-0355-4. Epub 2006 Nov 16.
Chloride channels are expressed along the entire mammalian nephron. They participate in transepithelial chloride transport, cell volume regulation and acidification of intracellular vesicles. Some chloride channels are constitutively active and others are regulated by either second messengers such as cAMP or Ca(++) or secondary to changes in membrane potential. The molecular identities of a number of chloride channels within the kidney are still unknown. Abnormalities in chloride channel expression and function in the kidney can cause a range of disorders such as autosomal recessive Dent's disease, Bartter's syndrome, renal tubular acidosis and diabetes insipidus. The purpose of this review is to give an overview of the chloride channels in the kidney and to focus on the function of renal chloride channels as revealed by diseases associated with channel dysfunction.
氯离子通道在整个哺乳动物肾单位中均有表达。它们参与跨上皮氯离子转运、细胞容积调节以及细胞内囊泡的酸化过程。一些氯离子通道持续处于激活状态,而其他通道则受诸如环磷酸腺苷(cAMP)或钙离子(Ca++)等第二信使调控,或继发于膜电位的变化。肾脏内许多氯离子通道的分子特性仍不明确。肾脏中氯离子通道表达及功能异常可引发一系列疾病,如常染色体隐性遗传性丹特病、巴特综合征、肾小管酸中毒及尿崩症。本综述旨在概述肾脏中的氯离子通道,并重点关注与通道功能障碍相关疾病所揭示的肾氯离子通道的功能。