A mild variant of cystic fibrosis.
作者信息
Alton E W
机构信息
Ion Transport Unit, National Heart and Lung Institute, London, UK.
出版信息
Thorax. 1996 Aug;51 Suppl 2(Suppl 2):S51-4. doi: 10.1136/thx.51.suppl_2.s51.
Abstract
摘要
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本文引用的文献
1
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.由于气道表面液体异常，囊性纤维化气道上皮细胞无法杀灭细菌。
Cell. 1996 Apr 19;85(2):229-36. doi: 10.1016/s0092-8674(00)81099-5.
2
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections.突变型囊性纤维化跨膜传导调节因子在囊性纤维化患者对肺部感染高度易感性中的作用。
Science. 1996 Jan 5;271(5245):64-7. doi: 10.1126/science.271.5245.64.
3
Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype.囊性纤维化患者轻度临床症状的决定因素。直肠活检中测得的残余氯分泌与基因型的关系。
J Clin Invest. 1994 Feb;93(2):461-6. doi: 10.1172/JCI116993.
4
Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface.囊性纤维化上皮细胞在其顶端表面有一个病原菌受体。
Proc Natl Acad Sci U S A. 1995 Mar 28;92(7):3019-23. doi: 10.1073/pnas.92.7.3019.
5
The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.囊性纤维化跨膜传导调节因子的常见变体被热休克蛋白70识别，并在前高尔基体非溶酶体区室中降解。
Proc Natl Acad Sci U S A. 1993 Oct 15;90(20):9480-4. doi: 10.1073/pnas.90.20.9480.
6
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.囊性纤维化中CFTR氯离子通道功能障碍的分子机制
Cell. 1993 Jul 2;73(7):1251-4. doi: 10.1016/0092-8674(93)90353-r.
7
Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.与具有改变的孔道特性的轻度疾病形式氯离子通道相关的囊性纤维化跨膜传导调节因子（CFTR）突变。
Nature. 1993 Mar 11;362(6416):160-4. doi: 10.1038/362160a0.
8
Delayed diagnosis of cystic fibrosis in children with a rare genotype (delta F508/R117H).罕见基因型（ΔF508/R117H）儿童囊性纤维化的延迟诊断
J Paediatr Child Health. 1995 Jun;31(3):168-71. doi: 10.1111/j.1440-1754.1995.tb00778.x.
9
CFTR as a cAMP-dependent regulator of sodium channels.囊性纤维化跨膜传导调节因子作为钠通道的环磷酸腺苷依赖性调节因子。
Science. 1995 Aug 11;269(5225):847-50. doi: 10.1126/science.7543698.
10
Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis.囊性纤维化患者呼吸上皮细胞跨膜生物电位差增加。
N Engl J Med. 1981 Dec 17;305(25):1489-95. doi: 10.1056/NEJM198112173052502.
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