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。
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections.
作者信息
Pier G B, Grout M, Zaidi T S, Olsen J C, Johnson L G, Yankaskas J R, Goldberg J B
机构信息
Channing Laboratory, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115-5899, USA.
出版信息
Science. 1996 Jan 5;271(5245):64-7. doi: 10.1126/science.271.5245.64.
Abstract
Cystic fibrosis (CF) patients are hypersusceptible to chronic Pseudomonas aeruginosa lung infections. Cultured human airway epithelial cells expressing the delta F508 allele of the cystic fibrosis transmembrane conductance regulator (CFTR) were defective in uptake of P. aeruginosa compared with cells expressing the wild-type allele. Pseudomonas aeruginosa lipopolysaccharide (LPS)-core oligosaccharide was identified as the bacterial ligand for epithelial cell ingestion; exogenous oligosaccharide inhibited bacterial ingestion in a neonatal mouse model, resulting in increased amounts of bacteria in the lungs. CFTR may contribute to a host-defense mechanism that is important for clearance of P. aeruginosa from the respiratory tract.
摘要
囊性纤维化（CF）患者极易受到铜绿假单胞菌慢性肺部感染的影响。与表达野生型等位基因的细胞相比，表达囊性纤维化跨膜传导调节因子（CFTR）的ΔF508等位基因的培养人气道上皮细胞在摄取铜绿假单胞菌方面存在缺陷。铜绿假单胞菌脂多糖（LPS）核心寡糖被确定为上皮细胞摄取的细菌配体；外源性寡糖在新生小鼠模型中抑制细菌摄取，导致肺部细菌数量增加。CFTR可能有助于一种对从呼吸道清除铜绿假单胞菌很重要的宿主防御机制。
相似文献
1
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.
2
How mutant CFTR may contribute to Pseudomonas aeruginosa infection in cystic fibrosis.突变型囊性纤维化跨膜传导调节因子（CFTR）如何导致囊性纤维化患者感染铜绿假单胞菌。
Am J Respir Crit Care Med. 1996 Oct;154(4 Pt 2):S175-82. doi: 10.1164/ajrccm/154.4_Pt_2.S175.
3
Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract.转基因囊性纤维化小鼠呼吸道中铜绿假单胞菌的早期清除率降低。
J Immunol. 2001 Jun 15;166(12):7410-8. doi: 10.4049/jimmunol.166.12.7410.
4
The role of the CFTR in susceptibility to Pseudomonas aeruginosa infections in cystic fibrosis.囊性纤维化跨膜传导调节因子在囊性纤维化患者对铜绿假单胞菌感染易感性中的作用。
Trends Microbiol. 2000 Nov;8(11):514-20. doi: 10.1016/s0966-842x(00)01872-2.
5
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections.囊性纤维化跨膜传导调节因子在对铜绿假单胞菌感染的固有免疫中的作用。
Proc Natl Acad Sci U S A. 2000 Aug 1;97(16):8822-8. doi: 10.1073/pnas.97.16.8822.
6
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.囊性纤维化跨膜传导调节因子（CFTR）突变与宿主对铜绿假单胞菌肺部感染的易感性
Curr Opin Microbiol. 2002 Feb;5(1):81-6. doi: 10.1016/s1369-5274(02)00290-4.
7
Asialo GM1 is a receptor for Pseudomonas aeruginosa adherence to regenerating respiratory epithelial cells.去唾液酸GM1是铜绿假单胞菌黏附于再生呼吸道上皮细胞的一种受体。
Infect Immun. 1996 May;64(5):1582-8. doi: 10.1128/iai.64.5.1582-1588.1996.
8
Mouse models of chronic lung infection with Pseudomonas aeruginosa: models for the study of cystic fibrosis.铜绿假单胞菌慢性肺部感染的小鼠模型：用于囊性纤维化研究的模型
Pediatr Pulmonol. 2000 Nov;30(5):413-24. doi: 10.1002/1099-0496(200011)30:5<413::aid-ppul8>3.0.co;2-9.
9
Relationship between cystic fibrosis respiratory tract bacterial communities and age, genotype, antibiotics and Pseudomonas aeruginosa.囊性纤维化呼吸道细菌群落与年龄、基因型、抗生素和铜绿假单胞菌的关系。
Environ Microbiol. 2010 May;12(5):1293-303. doi: 10.1111/j.1462-2920.2010.02173.x. Epub 2010 Feb 23.
10
Pseudomonas aeruginosa-induced apoptosis is defective in respiratory epithelial cells expressing mutant cystic fibrosis transmembrane conductance regulator.铜绿假单胞菌诱导的细胞凋亡在表达突变型囊性纤维化跨膜传导调节因子的呼吸道上皮细胞中存在缺陷。
Am J Respir Cell Mol Biol. 2003 Aug;29(2):188-97. doi: 10.1165/rcmb.4898.
引用本文的文献
1
Cross-membrane cooperation among bacteria can facilitate intracellular pathogenesis.细菌之间的跨膜合作可促进细胞内致病作用。
Nat Commun. 2025 Aug 11;16(1):7419. doi: 10.1038/s41467-025-62575-3.
2
Corticosteroids modulate biofilm formation and virulence of .皮质类固醇调节……的生物膜形成和毒力。 （原文此处不完整）
Biofilm. 2025 May 29;9:100289. doi: 10.1016/j.bioflm.2025.100289. eCollection 2025 Jun.
3
Evidence for intracellular .证据表明存在细胞内 。
J Bacteriol. 2024 May 23;206(5):e0010924. doi: 10.1128/jb.00109-24. Epub 2024 Apr 10.
4
The Inhibition of the Membrane-Bound Transcription Factor Site-1 Protease (MBTP1) Alleviates the p.Phe508del-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Defects in Cystic Fibrosis Cells.膜结合型转录因子位点 1 蛋白酶（MBTP1）的抑制减轻了囊性纤维化跨膜电导调节因子（CFTR）p.Phe508del 缺陷型囊性纤维化细胞的缺陷。
Cells. 2024 Jan 18;13(2):185. doi: 10.3390/cells13020185.
5
Reduced Sphingosine in Cystic Fibrosis Increases Susceptibility to Infections.囊性纤维化中鞘氨醇减少增加了感染易感性。
Int J Mol Sci. 2023 Sep 12;24(18):14004. doi: 10.3390/ijms241814004.
6
Current concepts on Pseudomonas aeruginosa interaction with human airway epithelium.当前关于铜绿假单胞菌与人呼吸道上皮相互作用的概念。
PLoS Pathog. 2023 Mar 30;19(3):e1011221. doi: 10.1371/journal.ppat.1011221. eCollection 2023 Mar.
7
and the Complement System: A Review of the Evasion Strategies.以及补体系统：逃避策略综述
Microorganisms. 2023 Mar 6;11(3):664. doi: 10.3390/microorganisms11030664.
8
The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis.CFTR 调节剂对囊性纤维化气道感染的影响。
Int J Mol Sci. 2022 Mar 23;23(7):3513. doi: 10.3390/ijms23073513.
9
The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis.未折叠蛋白反应、炎症与感染在囊性纤维化中的相互作用。
Cells. 2021 Nov 2;10(11):2980. doi: 10.3390/cells10112980.
10
Impact of Bacterial Toxins in the Lungs.细菌毒素对肺部的影响。
Toxins (Basel). 2020 Apr 2;12(4):223. doi: 10.3390/toxins12040223.
本文引用的文献
1
Nonmotility and phagocytic resistance of Pseudomonas aeruginosa isolates from chronically colonized patients with cystic fibrosis.从长期定植的囊性纤维化患者中分离出的铜绿假单胞菌菌株的非运动性和吞噬抗性。
Infect Immun. 1994 Feb;62(2):596-605. doi: 10.1128/iai.62.2.596-605.1994.
2
Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells.铜绿假单胞菌菌毛与去唾液酸GM1结合，去唾液酸GM1在囊性纤维化上皮细胞表面增加。
J Clin Invest. 1993 Oct;92(4):1875-80. doi: 10.1172/JCI116779.
3
Moderate stress protects female mice against bacterial infection of the bladder by eliciting uroepithelial shedding.适度应激通过引发尿路上皮脱落来保护雌性小鼠免受膀胱细菌感染。
Infect Immun. 1994 Dec;62(12):5505-10. doi: 10.1128/iai.62.12.5505-5510.1994.
4
Role of Pseudomonas aeruginosa pili in acute pulmonary infection.
Infect Immun. 1995 Apr;63(4):1278-85. doi: 10.1128/iai.63.4.1278-1285.1995.
5
Gonococcal rfaF mutants express Rd2 chemotype LPS and do not enter epithelial host cells.淋球菌rfaF突变体表达Rd2化学型脂多糖，且不进入上皮宿主细胞。
Mol Microbiol. 1995 Jan;15(2):267-75. doi: 10.1111/j.1365-2958.1995.tb02241.x.
6
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.
7
Differences in eucaryotic cell binding of Pseudomonas.假单胞菌在真核细胞结合方面的差异。
Microb Pathog. 1994 Nov;17(5):291-9. doi: 10.1006/mpat.1994.1075.
8
Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator.铜绿假单胞菌与囊性纤维化跨膜调节因子存在各种突变的患者的呼吸道上皮细胞的结合。
J Pediatr. 1995 Feb;126(2):230-3. doi: 10.1016/s0022-3476(95)70549-x.
9
The rfb locus from Pseudomonas aeruginosa strain PA103 promotes the expression of O antigen by both LPS-rough and LPS-smooth isolates from cystic fibrosis patients.铜绿假单胞菌PA103菌株的rfb基因座可促进来自囊性纤维化患者的粗糙型LPS和光滑型LPS分离株表达O抗原。
Mol Microbiol. 1994 Aug;13(3):427-34. doi: 10.1111/j.1365-2958.1994.tb00437.x.
10
The Pseudomonas aeruginosa algC gene encodes phosphoglucomutase, required for the synthesis of a complete lipopolysaccharide core.铜绿假单胞菌的algC基因编码磷酸葡萄糖变位酶，这是合成完整脂多糖核心所必需的。
J Bacteriol. 1994 Jun;176(12):3500-7. doi: 10.1128/jb.176.12.3500-3507.1994.
Zotero 插件