Role of CFTR in epithelial physiology.
Role of CFTR in epithelial physiology.
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DOI：
10.1007/s00018-016-2391-y
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发表时间：
2017-01
影响因子：
8
通讯作者：
Gray, Michael A.
中科院分区：
文献类型：
--
作者：
Saint-Criq, Vinciane;Gray, Michael A.
盐和液体的吸收和分泌是上皮功能和全身液体稳态的两个基本过程，因此在上皮组织中受到严格调节。CFTR阴离子通道在调节多种上皮组织（包括气道、GI和生殖道、汗腺和唾液腺）的分泌和吸收中起主要作用。因此，CFTR功能的缺陷与疾病有关也就不足为奇了，包括危及生命的分泌性腹泻，如霍乱，以及遗传性疾病，囊性纤维化（CF），这是高加索人群中最常见的限制生命的遗传疾病之一。最近，CFTR功能障碍也与急性胰腺炎、慢性阻塞性肺病（COPD）和哮喘高反应性的发病机制有关，强调了其在全身健康和疾病中的基本作用。CFTR调节上皮生理学中的许多机制，例如维持上皮表面水化和调节管腔pH。事实上，最近的研究已经确定管腔pH作为上皮屏障功能和先天防御的重要仲裁者，特别是在气道和胃肠道中。在本章中，我们将通过描述CFTR在三种不同组织（气道、胰腺和汗腺）中的特征来说明CFTR在上皮功能中的不同操作角色。
Salt and fluid absorption and secretion are two processes that are fundamental to epithelial function and whole body fluid homeostasis, and as such are tightly regulated in epithelial tissues. The CFTR anion channel plays a major role in regulating both secretion and absorption in a diverse range of epithelial tissues, including the airways, the GI and reproductive tracts, sweat and salivary glands. It is not surprising then that defects in CFTR function are linked to disease, including life-threatening secretory diarrhoeas, such as cholera, as well as the inherited disease, cystic fibrosis (CF), one of the most common life-limiting genetic diseases in Caucasian populations. More recently, CFTR dysfunction has also been implicated in the pathogenesis of acute pancreatitis, chronic obstructive pulmonary disease (COPD), and the hyper-responsiveness in asthma, underscoring its fundamental role in whole body health and disease. CFTR regulates many mechanisms in epithelial physiology, such as maintaining epithelial surface hydration and regulating luminal pH. Indeed, recent studies have identified luminal pH as an important arbiter of epithelial barrier function and innate defence, particularly in the airways and GI tract. In this chapter, we will illustrate the different operational roles of CFTR in epithelial function by describing its characteristics in three different tissues: the airways, the pancreas, and the sweat gland.
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DOI：
10.1085/jgp.200810097
发表时间：
2009-04
期刊：
The Journal of general physiology
影响因子：
--
作者：
Bertrand CA;Zhang R;Pilewski JM;Frizzell RA
通讯作者：
Frizzell RA
影响因子：
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作者：
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通讯作者：
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DOI：
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发表时间：
2006-05-15
影响因子：
24.7
作者：
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通讯作者：
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影响因子：
5.5
作者：
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影响因子：
29.4
作者：
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