Nielsen S, Agre P
Department of Cell Biology, University of Aarhus, Denmark.
Kidney Int. 1995 Oct;48(4):1057-68. doi: 10.1038/ki.1995.389.
The longstanding puzzle of membrane water permeability was advanced by the discovery of channel-forming integral protein (CHIP). This protein was shown to function as a water channel when expressed in Xenopus oocytes or when reconstituted into synthetic membranes. Site-directed mutagenesis and electron crystallography reveal tetrameric organization of CHIP, and the two halves of CHIP are tandem repeats folded into an obversely symmetric structure which resembles an hourglass. Each tetramer is comprised of functionally independent subunits. CHIP is the archetypal member of a newly-recognized family of membrane water transporters known as the "Aquaporins" (AQPs). AQP1 (CHIP) is abundant in the apical and basolateral membranes of renal proximal tubules and descending thin limbs, and is also present in a number of extra renal tissues. In the collecting duct, AQP2 is the predominant vasopressin-sensitive water channel. AQP2 is localized in the apical membrane and in intracellular vesicles which are targeted to the apical plasma membranes when stimulated by antidiuretic hormone. Humans are identified with mutations in AQP1 and AQP2 and exhibit contrasting clinical phenotypes. AQP3 resides in the basolateral membranes of collecting duct principal cells providing an exit pathway for water, and AQP4 is abundant in brain, where it apparently functions as the hypothalamic osmoreceptor responsible for secretion of antidiuretic hormone. Continued analysis of the aquaporins is providing detailed molecular insight into the fundamental physiological problems of water balance and water balance disorders.
通道形成整合蛋白(CHIP)的发现推动了长期存在的膜水通透性难题的研究进展。当这种蛋白在非洲爪蟾卵母细胞中表达或重组到合成膜中时,它被证明可作为水通道发挥作用。定点诱变和电子晶体学研究揭示了CHIP的四聚体结构,且CHIP的两半部分是串联重复序列,折叠成类似沙漏的反向对称结构。每个四聚体由功能独立的亚基组成。CHIP是新发现的一类膜水转运蛋白家族的原型成员,该家族被称为“水通道蛋白”(AQPs)。水通道蛋白1(CHIP)在肾近端小管和细段降支的顶端和基底外侧膜中含量丰富,也存在于许多肾外组织中。在集合管中,水通道蛋白2是主要的抗利尿激素敏感水通道。水通道蛋白2定位于顶端膜和细胞内囊泡中,当受到抗利尿激素刺激时,这些囊泡会靶向顶端质膜。人类已被鉴定出存在水通道蛋白1和水通道蛋白2的突变,并表现出截然不同的临床表型。水通道蛋白3存在于集合管主细胞的基底外侧膜中,为水提供了一条排出途径,而水通道蛋白4在大脑中含量丰富,它显然在大脑中作为下丘脑渗透压感受器发挥作用,负责抗利尿激素的分泌。对水通道蛋白的持续分析正在为水平衡和水平衡紊乱等基本生理问题提供详细的分子见解。