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Being positive: revisiting the elevated sodium permeability hypothesis in cystic fibrosis.

作者信息

Kirk Kevin L

机构信息

Department of Cell, Developmental and Integrative Biology, and Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham,1720 2nd Avenue South, Birmingham, AL35294-0005, USA.

出版信息

J Physiol. 2013 Aug 1;591(15):3675-6. doi: 10.1113/jphysiol.2013.260406.

DOI:10.1113/jphysiol.2013.260406
PMID:23908409
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3752447/
Abstract
摘要

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Being positive: revisiting the elevated sodium permeability hypothesis in cystic fibrosis.积极看待:重新审视囊性纤维化中钠通透性升高假说
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Increased apical Na+ permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport.囊性纤维化中顶端 Na+通透性增加得到上皮离子转运定量模型的支持。
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[Determination of nasal transepithelial potential difference (DDPTE) in cystic fibrosis. Analysis of a simplified measurement technique].[囊性纤维化中鼻跨上皮电位差(DDPTE)的测定。一种简化测量技术的分析]
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Cystic fibrosis and non-cystic-fibrosis human nasal epithelium show analogous Na+ absorption and reversible block by phenamil.囊性纤维化和非囊性纤维化的人类鼻上皮显示出类似的钠离子吸收以及苯甲脒的可逆性阻断作用。
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Association of cystic fibrosis transmembrane conductance regulator with epithelial sodium channel subunits carrying Liddle's syndrome mutations.囊性纤维化跨膜电导调节因子与携带利德尔综合征突变的上皮钠通道亚基的关联。
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本文引用的文献

1
Increased apical Na+ permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport.囊性纤维化中顶端 Na+通透性增加得到上皮离子转运定量模型的支持。
J Physiol. 2013 Aug 1;591(15):3681-92. doi: 10.1113/jphysiol.2013.253955. Epub 2013 Jun 3.
2
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance.人类囊性纤维化气道上皮细胞的氯离子电导降低,但钠离子电导没有增加。
Proc Natl Acad Sci U S A. 2011 Jun 21;108(25):10260-5. doi: 10.1073/pnas.1106695108. Epub 2011 Jun 6.
3
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia.新生猪囊性纤维化气道上皮细胞中阴离子转运丧失而不伴有钠离子吸收增加。
Cell. 2010 Dec 10;143(6):911-23. doi: 10.1016/j.cell.2010.11.029.
4
ENaC-CFTR interactions: the role of electrical coupling of ion fluxes explored in an epithelial cell model.上皮钠通道-囊性纤维化跨膜传导调节因子相互作用:在一个上皮细胞模型中对离子通量电偶联作用的探索
Pflugers Arch. 2003 Jan;445(4):522-8. doi: 10.1007/s00424-002-0956-0. Epub 2002 Dec 4.
5
Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures.囊性纤维化人类原代细胞培养物中氯离子通透性降低和钠离子通透性增加的证据。
J Physiol. 1988 Nov;405:77-103. doi: 10.1113/jphysiol.1988.sp017322.