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1
The biochemical defect in cystic fibrosis.囊性纤维化的生化缺陷。
J R Soc Med. 1992;85 Suppl 19(Suppl 19):2-5.
2
Toward defining the function of the cystic fibrosis gene product.
J Pediatr Gastroenterol Nutr. 1991 Nov;13(4):432-3. doi: 10.1097/00005176-199111000-00019.
3
The basic defect in cystic fibrosis.囊性纤维化的基本缺陷。
Trends Biochem Sci. 1991 Dec;16(12):474-7. doi: 10.1016/0968-0004(91)90183-v.
4
Cystic fibrosis transmembrane conductance regulator (CFTR).囊性纤维化跨膜传导调节因子(CFTR)
Br Med Bull. 1992 Oct;48(4):754-65. doi: 10.1093/oxfordjournals.bmb.a072576.
5
Towards the biochemical defect in cystic fibrosis.关于囊性纤维化的生化缺陷
Lancet. 1989 Dec 16;2(8677):1433.
6
Probing the basic defect in cystic fibrosis.探究囊性纤维化的基本缺陷。
Curr Opin Genet Dev. 1991 Jun;1(1):4-10. doi: 10.1016/0959-437x(91)80032-h.
7
Cystic fibrosis. Sickly channels in mild disease.囊性纤维化。轻度疾病中的功能异常通道。
Nature. 1993 Mar 11;362(6416):106. doi: 10.1038/362106a0.
8
Outwardly rectifying chloride channels and CF: a divorce and remarriage.外向整流性氯离子通道与囊性纤维化:一段分离与再结合的历程
J Bioenerg Biomembr. 1993 Feb;25(1):27-35. doi: 10.1007/BF00768065.
9
Cystic fibrosis, the CFTR, and rectifying Cl- channels.
Adv Exp Med Biol. 1991;290:253-69; discussion 269-72. doi: 10.1007/978-1-4684-5934-0_25.
10
Correction of the apical membrane chloride permeability defect in polarized cystic fibrosis airway epithelia following retroviral-mediated gene transfer.逆转录病毒介导的基因转移后极化囊性纤维化气道上皮细胞顶端膜氯化物通透性缺陷的纠正
Hum Gene Ther. 1992 Jun;3(3):253-66. doi: 10.1089/hum.1992.3.3-253.

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New insights into the pathogenesis of intestinal dysfunction: secretory diarrhea and cystic fibrosis.肠道功能障碍发病机制的新见解:分泌性腹泻与囊性纤维化。
World J Gastroenterol. 2000 Aug;6(4):470-474. doi: 10.3748/wjg.v6.i4.470.
2
Ion-transporting activity in the murine colonic epithelium of normal animals and animals with cystic fibrosis.正常动物和患有囊性纤维化的动物的小鼠结肠上皮中的离子转运活性。
Pflugers Arch. 1994 Oct;428(5-6):508-15. doi: 10.1007/BF00374572.

本文引用的文献

1
Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease.胰腺囊性纤维化患者汗液中电解质成分异常;临床意义及其与疾病的关系。
Pediatrics. 1953 Nov;12(5):549-63.
2
Chloride impermeability in cystic fibrosis.囊性纤维化中的氯离子不渗透性
Nature. 1983 Feb 3;301(5899):421-2. doi: 10.1038/301421a0.
3
Abnormal ion permeation through cystic fibrosis respiratory epithelium.异常离子透过囊性纤维化呼吸道上皮。
Science. 1983 Sep 9;221(4615):1067-70. doi: 10.1126/science.6308769.
4
Abnormal apical cell membrane in cystic fibrosis respiratory epithelium. An in vitro electrophysiologic analysis.囊性纤维化呼吸道上皮细胞顶端细胞膜异常。一项体外电生理分析。
J Clin Invest. 1987 Jan;79(1):80-5. doi: 10.1172/JCI112812.
5
Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation.囊性纤维化呼吸道上皮细胞中的钠离子转运。基础速率异常及对腺苷酸环化酶激活的反应。
J Clin Invest. 1986 Nov;78(5):1245-52. doi: 10.1172/JCI112708.
6
Abnormal respiratory epithelial ion transport in cystic fibrosis.囊性纤维化中异常的呼吸道上皮离子转运。
Clin Chest Med. 1986 Jun;7(2):285-97.
7
Identification of the cystic fibrosis gene: chromosome walking and jumping.囊性纤维化基因的鉴定:染色体步移与跳跃
Science. 1989 Sep 8;245(4922):1059-65. doi: 10.1126/science.2772657.
8
Identification of the cystic fibrosis gene: genetic analysis.囊性纤维化基因的鉴定:遗传分析
Science. 1989 Sep 8;245(4922):1073-80. doi: 10.1126/science.2570460.
9
Cystic fibrosis affects chloride and sodium channels in human airway epithelia.囊性纤维化影响人类气道上皮细胞中的氯离子和钠离子通道。
Can J Physiol Pharmacol. 1989 Oct;67(10):1362-5. doi: 10.1139/y89-217.
10
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.囊性纤维化基因的鉴定:互补DNA的克隆与特性分析
Science. 1989 Sep 8;245(4922):1066-73. doi: 10.1126/science.2475911.

The biochemical defect in cystic fibrosis.

作者信息

Cuthbert A W

机构信息

Department of Pharmacology, University of Cambridge.

出版信息

J R Soc Med. 1992;85 Suppl 19(Suppl 19):2-5.

PMID:1375960
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1295445/
Abstract
摘要