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囊性纤维化中的液体和电解质吸收状况。

Status of fluid and electrolyte absorption in cystic fibrosis.

机构信息

Department of Pediatrics, UCSD School of Medicine, University of California, San Diego, La Jolla, CA 92093, USA.

出版信息

Cold Spring Harb Perspect Med. 2013 Jan 1;3(1):a009555. doi: 10.1101/cshperspect.a009555.

Abstract

Salt and fluid absorption is a shared function of many of the body's epithelia, but its use is highly adapted to the varied physiological roles of epithelia-lined organs. These functions vary from control of hydration of outward-facing epithelial surfaces to conservation and regulation of total body volume. In the most general context, salt and fluid absorption is driven by active Na(+) absorption. Cl(-) is absorbed passively through various available paths in response to the electrical driving force that results from active Na(+) absorption. Absorption of salt creates a concentration gradient that causes water to be absorbed passively, provided the epithelium is water permeable. Key differences notwithstanding, the transport elements used for salt and fluid absorption are broadly similar in diverse epithelia, but the regulation of these elements enables salt absorption to be tailored to very different physiological needs. Here we focus on salt absorption by exocrine glands and airway epithelia. In cystic fibrosis, salt and fluid absorption by gland duct epithelia is effectively prevented by the loss of cystic fibrosis transmembrane conductance regulator (CFTR). In airway epithelia, salt and fluid absorption persists, in the absence of CFTR-mediated Cl(-) secretion. The contrast of these tissue-specific changes in CF tissues is illustrative of how salt and fluid absorption is differentially regulated to accomplish tissue-specific physiological objectives.

摘要

盐和液体吸收是许多身体上皮细胞的共同功能,但它的使用高度适应于上皮衬里器官的各种生理作用。这些功能从控制面向外的上皮表面的水合作用到保持和调节全身体积不等。从最一般的角度来看,盐和液体的吸收是由主动 Na(+)吸收驱动的。Cl(-)通过各种可用途径被动吸收,以响应主动 Na(+)吸收产生的电驱动力。盐的吸收会产生浓度梯度,从而使水被动吸收,只要上皮是可渗透水的。尽管存在关键差异,但不同上皮细胞中用于盐和液体吸收的运输元素广泛相似,但这些元素的调节使盐吸收能够适应非常不同的生理需求。在这里,我们重点关注外分泌腺和气道上皮细胞的盐吸收。在囊性纤维化中,由于囊性纤维化跨膜电导调节因子 (CFTR) 的丧失,腺体导管上皮的盐和液体吸收被有效阻止。在气道上皮细胞中,即使没有 CFTR 介导的 Cl(-)分泌,盐和液体吸收也会持续存在。CF 组织中这些组织特异性变化的对比说明了盐和液体吸收是如何被差异化调节以实现组织特异性生理目标的。

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