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Journal of Cystic Fibrosis最新文献

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Insights into epithelial-mesenchymal transition from cystic fibrosis rat models. 从囊性纤维化大鼠模型中了解上皮-间质转化。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-11 DOI: 10.1016/j.jcf.2024.09.003
Nathan Rout-Pitt, Bernadette Boog, Alexandra McCarron, Nicole Reyne, David Parsons, Martin Donnelley
{"title":"Insights into epithelial-mesenchymal transition from cystic fibrosis rat models.","authors":"Nathan Rout-Pitt, Bernadette Boog, Alexandra McCarron, Nicole Reyne, David Parsons, Martin Donnelley","doi":"10.1016/j.jcf.2024.09.003","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.09.003","url":null,"abstract":"<p><strong>Background: </strong>Molecular pathways contributing to Cystic Fibrosis pathogenesis remain poorly understood. Epithelial-mesenchymal transition (EMT) has been recently observed in CF lungs and certain CFTR mutation classes may be more susceptible than others. No investigations of EMT processes in CF animal models have been reported.</p><p><strong>Aim: </strong>The aim of this study was to assess the expression of EMT-related markers in Phe508del and knockout (CFTR-KO) rat lung tissue and tracheal-derived basal epithelial stem cells, to determine whether CFTR dysfunction can produce an EMT state.</p><p><strong>Method: </strong>The expression of EMT-related markers in lung tissue and cultured tracheal basal epithelial stem cells from wildtype (WT), Phe508del, and CFTR-KO rats were assessed using qPCR and Western blots. Cell responses were evaluated in the presence of Rho-associated protein kinase (ROCK) inhibitor Y27632, which blocks EMT-pathways, or after treatment with TGFβ1 to stimulate EMT.</p><p><strong>Results: </strong>Different gene expression profiles were observed between Phe508del and CFTR-KO rat models compared to wild type. There was lower expression of type 1 collagen in KO lungs and primary cell cultures, while Phe508del lungs and cells had higher expression, particularly when treated with TGFβ1. The addition of Y27632 rescued changes in EMT related genes in Phe508del cells but not in KO cells.</p><p><strong>Conclusion: </strong>Our findings show the first evidence of upregulated EMT pathways in the lungs and airway cells of any CF animal model. Differences in the regulation of the EMT genes and proteins in the Phe508del and CFTR-KO cells suggest that the signalling pathways underlying EMT are CFTR mutation dependent.</p>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142288301","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Letter to the Editor. 致编辑的信
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-06 DOI: 10.1016/j.jcf.2024.08.006
Atefeh Khajeh, Yakup Kilic, Rushabh Shah, Simon Pg Padley, Carole A Ridge
{"title":"Letter to the Editor.","authors":"Atefeh Khajeh, Yakup Kilic, Rushabh Shah, Simon Pg Padley, Carole A Ridge","doi":"10.1016/j.jcf.2024.08.006","DOIUrl":"https://doi.org/10.1016/j.jcf.2024.08.006","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":" ","pages":""},"PeriodicalIF":5.4,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142145742","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Are demographic findings really possible from reports in a spontaneous reporting system? 自发报告系统中的报告真的能得出人口统计结果吗？
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/j.jcf.2024.07.007
Yoshihiro Noguchi, Tomoaki Yoshimura
{"title":"Are demographic findings really possible from reports in a spontaneous reporting system?","authors":"Yoshihiro Noguchi,&nbsp;Tomoaki Yoshimura","doi":"10.1016/j.jcf.2024.07.007","DOIUrl":"10.1016/j.jcf.2024.07.007","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 5","pages":"Page 1031"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141734261","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of interruption of CFTR modulator therapies CFTR 调节器疗法中断的影响。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/j.jcf.2024.05.006
{"title":"Impact of interruption of CFTR modulator therapies","authors":"","doi":"10.1016/j.jcf.2024.05.006","DOIUrl":"10.1016/j.jcf.2024.05.006","url":null,"abstract":"<div><p>Novel drug therapy targeting the defective cystic fibrosis transmembrane conductance regulator protein has the potential to significantly enhance the quality of life for numerous patients with cystic fibrosis. However, in some countries social insurance does not pay for modulators because these drugs are extremely expensive. This study sought to understand the impact on the health of children whose modulator treatments were interrupted because of legal procedures and delivery processes. Our study identified that the significant increase in percent-predicted forced expiratory volume levels (FEV1) and BMI z-score values associated with modulator therapies decreased sharply with their discontinuation. Significant worsening in FEV1, BMI z-scores, and BW z-scores were detected in the first follow-up visit after therapy discontinuation within 1 month. Eight patients had a reduction of FEV1 of more than 10%. The findings suggest that modulatory treatment continuation is important, and it is crucial that treatment is not interrupted.</p></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 5","pages":"Pages 947-949"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140957500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
42 BOS-318 attenuates Pseudomonas aeruginosa lung infection in mouse models 42 BOS-318 可减轻小鼠模型中铜绿假单胞菌的肺部感染
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/S1569-1993(24)00885-3
O. Bernard, M. Kwon, M. Magnen, M. Yu
{"title":"42 BOS-318 attenuates Pseudomonas aeruginosa lung infection in mouse models","authors":"O. Bernard,&nbsp;M. Kwon,&nbsp;M. Magnen,&nbsp;M. Yu","doi":"10.1016/S1569-1993(24)00885-3","DOIUrl":"10.1016/S1569-1993(24)00885-3","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S22-S23"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142135121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
32 Pseudomonas aeruginosa detects fungal toxins using a novel regulatory cascade 32 铜绿假单胞菌利用新型调控级联检测真菌毒素
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/S1569-1993(24)00875-0
J. Aycock, S. Dolan
{"title":"32 Pseudomonas aeruginosa detects fungal toxins using a novel regulatory cascade","authors":"J. Aycock,&nbsp;S. Dolan","doi":"10.1016/S1569-1993(24)00875-0","DOIUrl":"10.1016/S1569-1993(24)00875-0","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Page S17"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142135963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
49 Differential effects of cationic antimicrobials on intrinsic antibiotic resistance in Pseudomonas aeruginosa 49 阳离子抗菌剂对铜绿假单胞菌内在抗生素耐药性的不同影响
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/S1569-1993(24)00892-0
J. Albin, D. Vithanage, C. Johnson, B. Pentelute
{"title":"49 Differential effects of cationic antimicrobials on intrinsic antibiotic resistance in Pseudomonas aeruginosa","authors":"J. Albin,&nbsp;D. Vithanage,&nbsp;C. Johnson,&nbsp;B. Pentelute","doi":"10.1016/S1569-1993(24)00892-0","DOIUrl":"10.1016/S1569-1993(24)00892-0","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S26-S27"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136125","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
73 Treatment outcomes in nontuberculous mycobacterial infection in people with cystic fibrosis in Denmark, 2012–2022 73 2012-2022 年丹麦囊性纤维化患者非结核分枝杆菌感染的治疗结果
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/S1569-1993(24)00916-0
C. Leo-Hansen, T. Bryrup, M. Jeppesen, M. Kolpen, P. Jensen, T. Pressler, H. Johansen, D. Faurholt-Jepsen, M. Olsen, T. Qvist
{"title":"73 Treatment outcomes in nontuberculous mycobacterial infection in people with cystic fibrosis in Denmark, 2012–2022","authors":"C. Leo-Hansen,&nbsp;T. Bryrup,&nbsp;M. Jeppesen,&nbsp;M. Kolpen,&nbsp;P. Jensen,&nbsp;T. Pressler,&nbsp;H. Johansen,&nbsp;D. Faurholt-Jepsen,&nbsp;M. Olsen,&nbsp;T. Qvist","doi":"10.1016/S1569-1993(24)00916-0","DOIUrl":"10.1016/S1569-1993(24)00916-0","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Pages S39-S40"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
58 CbrA mediates Pseudomonas aeruginosa antagonism of Candida albicans 58 CbrA 介导铜绿假单胞菌对白色念珠菌的拮抗作用
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/S1569-1993(24)00901-9
A. Conaway, I. Todorovic, D. Mould, D. Hogan
{"title":"58 CbrA mediates Pseudomonas aeruginosa antagonism of Candida albicans","authors":"A. Conaway,&nbsp;I. Todorovic,&nbsp;D. Mould,&nbsp;D. Hogan","doi":"10.1016/S1569-1993(24)00901-9","DOIUrl":"10.1016/S1569-1993(24)00901-9","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Page S32"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
60 Pyochelin-mediated cefiderocol sensitization depends on the FptA transporter 60 Pyochelin 介导的头孢哌酮致敏作用取决于 FptA 转运体
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2024-09-01 DOI: 10.1016/S1569-1993(24)00903-2
A. Milesi Galdino, P. Jorth
{"title":"60 Pyochelin-mediated cefiderocol sensitization depends on the FptA transporter","authors":"A. Milesi Galdino,&nbsp;P. Jorth","doi":"10.1016/S1569-1993(24)00903-2","DOIUrl":"10.1016/S1569-1993(24)00903-2","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 ","pages":"Page S33"},"PeriodicalIF":5.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142136534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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