Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.

Ostedgaard LS; Randak C; Rokhlina T; Karp P; Vermeer D; Ashbourne Excoffon KJ; Welsh MJ

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Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.

机译：C端缺失对囊性纤维化气道上皮细胞中囊性纤维化跨膜电导调节器功能的影响。

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摘要

To better understand the function of the conserved C terminus of the cystic fibrosis (CF) transmembrane conductance regulator, we studied constructs containing deletions in the C-terminal tail. When expressed in well differentiated CF airway epithelia, each construct localized predominantly to the apical membrane and generated transepithelial Cl(-) current. The results suggested that neither the C-terminal PSD-95Discs-largeZO-1 (PDZ)-interacting motif nor other C-terminal sequences were absolutely required for apical expression in airway epithelia. Surprisingly, deleting an acidic cluster near the C terminus reduced both channel opening rate and transepithelial Cl(-) transport, indicating that it influences channel gating. These results may help explain the relative paucity of CF-associated mutations in the C terminus.

机译：为了更好地了解囊性纤维化（CF）跨膜电导调节剂的保守C末端的功能，我们研究了在C末端尾巴中含有缺失的构建体。当在分化良好的CF气道上皮细胞中表达时，每种构建体主要定位于心尖膜并产生跨上皮Cl（-）电流。结果表明，C端PSD-95Discs-largeZO-1（PDZ）相互作用的基序和其他C端序列对于在气道上皮中的根尖表达绝对不是必需的。出人意料的是，删除C末端附近的酸性集群减少通道开放率和跨上皮Cl（-）传输，表明它影响通道门控。这些结果可能有助于解释C末端CF相关突变相对较少。

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来源
《Proceedings of the National Academy of Sciences of the United States of America》 |2003年第4期|P.1937-1942|共6页
作者
Ostedgaard LS; Randak C; Rokhlina T; Karp P; Vermeer D; Ashbourne Excoffon KJ; Welsh MJ;
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作者单位

Howard Hughes Medical Institute, Departments of Internal Medicine and Physiology and Biophysics, University of Iowa, Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242.;

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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
关键词
Carbon ion; Cystic Fibrosis; physiological aspects; Chloride measurement; 囊性纤维变性; 囊性纤维化穿膜传导调节因子;

机译：Carbon ion;Cystic Fibrosis;physiological aspects;Chloride measurement;囊性纤维变性;囊性纤维化穿膜传导调节因子;

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1. Adenovirus 5-fiber 35 chimeric vector mediates efficient apical correction of the cystic fibrosis transmembrane conductance regulator defect in cystic fibrosis primary airway epithelia. [J] . Granio O, Ashbourne Excoffon KJ, Henning P, Human gene therapy . 2010,第3期

机译：腺病毒5纤维35嵌合载体介导囊性纤维化原发性气道上皮细胞中的囊性纤维化跨膜电导调节因子缺陷的有效根尖矫正。
2. Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. [J] . G M Denning, L S Ostedgaard, M J Welsh Journal of cell biology . 1992,第3期

机译：囊性纤维化气道上皮细胞原代培养中囊性纤维化跨膜电导调节剂的异常定位。
3. Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections [J] . Katherine B. Hisert, Sonya L. Heltshe, Christopher Pope, American journal of respiratory and critical care medicine . 2017,第12期

机译：恢复囊性纤维化跨膜电导调节器功能可减少囊性纤维化和慢性肺部感染患者的气道细菌和炎症
4. Cystic fibrosis transmembrane conductance regulator gene mutations cause 'black' pigment gallstone formation: new insights from mouse models and implications for therapeutic interventions in cystic fibrosis [C] . A. L. BRODERICK, H. WITTENBURG, M. A. LYONS, Falk Symposium . 2004

机译：囊性纤维化跨膜电导调节剂基因突变引起'黑色'颜料胆结石形成：来自小鼠模型的新见解，以及对囊性纤维化治疗干预的影响
5. Treating an N-glycosylation abnormality in airways with a cystic fibrosis transmembrane conductance regulator protein deficiency reduces Pseudomonas aeruginosa colonization; A hallmark symptom of cystic fibrosis [D] . Martino, Ashley Thomas 2007

机译：用囊性纤维化跨膜电导调节蛋白缺乏治疗气道N-糖基化异常可减少铜绿假单胞菌定植；囊性纤维化的典型症状
6. Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia [O] . Lynda S. Ostedgaard, Christoph Randak, Tatiana Rokhlina, 2003

机译：C端缺失对囊性纤维化气道上皮细胞囊性纤维化跨膜电导调节子功能的影响
7. Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia [O] . Ostedgaard, Lynda S., Randak, Christoph, Rokhlina, Tatiana, 2003

机译：C端缺失对囊性纤维化气道上皮细胞囊性纤维化跨膜电导调节子功能的影响

Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia.

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