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Items: 1 to 20 of 359

1.
The anion exchanger slc26a3 regulates colonic mucus expansion during steady state and in response to prostaglandin E2, while Cftr regulates de novo mucus release in response to carbamylcholine
Penny L. Ljungholm, Anna Ermund, Molly M. Söderlund Garsveden, Victor L. Pettersson, Jenny K. Gustafsson
Pflugers Arch. 2024; 476(8): 1209–1219. Published online 2024 Jun 3. doi: 10.1007/s00424-024-02975-4
PMCID:
PMC11271379
AbstractArticlePDF–5.6M
2.
Respiratory syncytial virus persistent infection causes acquired CFTR dysfunction in human bronchial epithelial cells
LONG Chunjiao, QI Mingming, WANG Jinmei, LUO Jinhua, QIN Xiaoqun, GAO Ge, XIANG Yang
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2021 Sep 28; 46(9): 949–957. doi: 10.11817/j.issn.1672-7347.2021.210210
PMCID:
PMC10930179
AbstractArticlePDF–1.6M
3.
CTNNAL1 deficiency suppresses CFTR expression in HDM-induced asthma mouse model through ROCK1-CAL signaling pathway: CTNNAL1 increases CFTR expression in asthma mouse model
Di Wu, Jiahui Zhu, Fang Yang, Riwang Li, Lexin Liu, Dahai Liu, Chi Liu, Xiangping Qu, Huijun Liu, Ming Ji, Xiaoqun Qin, Lan Hua, Yang Xiang
Acta Biochim Biophys Sin (Shanghai) 2023 Oct 25; 55(10): 1618–1629. Published online 2023 Sep 15. doi: 10.3724/abbs.2023152
PMCID:
PMC10579809
AbstractArticlePDF–3.3M
4.
Rhox8 homeobox gene ablation leads to rete testis abnormality and male subfertility in mice
Yeongseok Oh, Maho Kasu, Constence J Bottoms, Jenna C Douglas, Nikola Sekulovski, Kanako Hayashi, James A MacLean II
Biol Reprod. 2023 Oct; 109(4): 520–532. Published online 2023 Jul 20. doi: 10.1093/biolre/ioad077
PMCID:
PMC10577278
AbstractArticlePDF–1.9M
5.
Epithelial TNF controls cell differentiation and CFTR activity to maintain intestinal mucin homeostasis
Efren A. Reyes, David Castillo-Azofeifa, Jérémie Rispal, Tomas Wald, Rachel K. Zwick, Brisa Palikuqi, Angela Mujukian, Shervin Rabizadeh, Alexander R. Gupta, James M. Gardner, Dario Boffelli, Zev J. Gartner, Ophir D. Klein
J Clin Invest. 2023 Oct 16; 133(20): e163591. Published online 2023 Oct 16. doi: 10.1172/JCI163591
PMCID:
PMC10575728
AbstractArticlePDF–4.7M
6.
CFTR Modulates Hypothalamic Neuron Excitability to Maintain Female Cycle
Yong Wu, Yanting Que, Junjiang Chen, Lei Sun, Jinghui Guo, Ye Chun Ruan
Int J Mol Sci. 2023 Aug; 24(16): 12572. Published online 2023 Aug 8. doi: 10.3390/ijms241612572
PMCID:
PMC10454438
AbstractArticlePDF–2.5M
7.
CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity
Elizabeth L. Kramer, Kristin M. Hudock, Cynthia R. Davidson, John P. Clancy
Respir Res. 2023; 24: 198. Published online 2023 Aug 11. doi: 10.1186/s12931-023-02495-2
PMCID:
PMC10416378
AbstractArticlePDF–2.9M
8.
TRIM28 modulates nuclear receptor signaling to regulate uterine function
Rong Li, Tianyuan Wang, Ryan M. Marquardt, John P. Lydon, San-Pin Wu, Francesco J. DeMayo
Nat Commun. 2023; 14: 4605. Published online 2023 Aug 1. doi: 10.1038/s41467-023-40395-7
PMCID:
PMC10393996
AbstractArticlePDF–11M
9.
CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway
Kyung-Mi Choi, Sung-Hee Cho, Jung Hak Kim, Ae-Rhee Lilian Kim, Xiangmudong Kong, John C. Yoon
J Cyst Fibros. Author manuscript; available in PMC 2023 Jul 10.
Published in final edited form as: J Cyst Fibros. 2023 Jan; 22(1): 132–139. Published online 2022 Sep 8. doi: 10.1016/j.jcf.2022.08.012
PMCID:
PMC10332805
AbstractArticlePDF–1.5M
10.
In Vivo Inflammation Caused by Achromobacter spp. Cystic Fibrosis Clinical Isolates Exhibiting Different Pathogenic Characteristics
Angela Sandri, Giulia Maria Saitta, Laura Veschetti, Federico Boschi, Rebeca Passarelli Mantovani, Maria Carelli, Paola Melotti, Caterina Signoretto, Marzia Boaretti, Giovanni Malerba, Maria M. Lleò
Int J Mol Sci. 2023 Apr; 24(8): 7432. Published online 2023 Apr 18. doi: 10.3390/ijms24087432
PMCID:
PMC10139000
AbstractArticlePDF–2.9M
11.
Loss-of-function CFTR p.G970D missense mutation might cause congenital bilateral absence of the vas deferens and be associated with impaired spermatogenesis
Jian-Wen Hou, Xiao-Liang Li, Li Wang, Cong-Ling Dai, Na Li, Xiao-Hui Jiang, Yue-Qiu Tan, Er-Po Tian, Qin-Tong Li, Wen-Ming Xu
Asian J Androl. 2023 Jan-Feb; 25(1): 58–65. Published online 2022 Jun 3. doi: 10.4103/aja202236
PMCID:
PMC9933970
AbstractArticlePDF–4.9M
12.
Linaclotide improves gastrointestinal transit in cystic fibrosis mice by inhibiting sodium/hydrogen exchanger 3
Daniel R. McHugh, Calvin U. Cotton, Fraser J. Moss, Megan Vitko, Dana M. Valerio, Thomas J. Kelley, Shuyu Hao, Anjum Jafri, Mitchell L. Drumm, Walter F. Boron, Robert C. Stern, Kimberly McBennett, Craig A. Hodges
Am J Physiol Gastrointest Liver Physiol. 2018 Nov 1; 315(5): G868–G878. Published online 2018 Aug 17. doi: 10.1152/ajpgi.00261.2017
PMCID:
PMC9925117
AbstractArticle
13.
Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis
Hasan H. Öz, Ee-Chun Cheng, Caterina Di Pietro, Toma Tebaldi, Giulia Biancon, Caroline Zeiss, Ping-Xia Zhang, Pamela H. Huang, Sofia S. Esquibies, Clemente J. Britto, Jonas C Schupp, Thomas S. Murray, Stephanie Halene, Diane S. Krause, Marie E. Egan, Emanuela M. Bruscia
Cell Rep. Author manuscript; available in PMC 2023 Jan 11.
Published in final edited form as: Cell Rep. 2022 Dec 13; 41(11): 111797. doi: 10.1016/j.celrep.2022.111797
PMCID:
PMC9833830
AbstractArticlePDF–1.7M
14.
Elexacaftor/Tezacaftor/Ivacaftor Disrupts Respiratory Tract Development in a Murine Fetal Lung Explant Model
Mickaël Lhuillier, Laura Aoust, Elise Dreano, Marie-Laure Franco-Montoya, Kim Landry-Truchon, Nicolas Houde, Stéphanie Chhun, Alexandre Hinzpeter, Aleksander Edelman, Christophe Delacourt, Lucie Jeannotte, Isabelle Sermet-Gaudelus, Alice Hadchouel
Am J Respir Cell Mol Biol. 2022 Dec 1; 67(6): 723–726. Published online 2022 Dec 1. doi: 10.1165/rcmb.2022-0175LE
PMCID:
PMC9743190
ArticlePDF–686K
15.
Coxsackievirus B infections are common in Cystic Fibrosis and experimental evidence supports protection by vaccination
Virginia M. Stone, Renata Utorova, Marta Butrym, Amir-Babak Sioofy-Khojine, Minna M. Hankaniemi, Emma E. Ringqvist, Marfa Blanter, Anirudra Parajuli, Terezia Pincikova, Björn Fischler, Ferenc Karpati, Vesa P. Hytönen, Heikki Hyöty, Lena Hjelte, Malin Flodström-Tullberg
iScience. 2022 Oct 21; 25(10): 105070. Published online 2022 Sep 5. doi: 10.1016/j.isci.2022.105070
PMCID:
PMC9490033
AbstractArticlePDF–3.3M
16.
The NHE3 Inhibitor Tenapanor Prevents Intestinal Obstructions in CFTR-Deleted Mice
Xinjie Tan, Archana Kini, Dorothee Römermann, Ursula Seidler
Int J Mol Sci. 2022 Sep; 23(17): 9993. Published online 2022 Sep 1. doi: 10.3390/ijms23179993
PMCID:
PMC9456459
AbstractArticlePDF–6.1M
17.
Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosis
Caterina Di Pietro, Hasan H. Öz, Ping-xia Zhang, Ee-chun Cheng, Valentino Martis, Tracey L. Bonfield, Thomas J. Kelley, Ronald Jubin, Abraham Abuchowski, Diane S. Krause, Marie E. Egan, Thomas S. Murray, Emanuela M. Bruscia
Exp Mol Med. 2022 May; 54(5): 639–652. Published online 2022 May 17. doi: 10.1038/s12276-022-00770-8
PMCID:
PMC9166813
AbstractArticlePDF–5.8M
18.
Treatment With LAU-7b Complements CFTR Modulator Therapy by Improving Lung Physiology and Normalizing Lipid Imbalance Associated With CF Lung Disease
Amanda Centorame, Daciana Catalina Dumut, Mina Youssef, Martin Ondra, Irenej Kianicka, Juhi Shah, Radu Alexandru Paun, Tomas Ozdian, John W. Hanrahan, Ekaterina Gusev, Basil Petrof, Marian Hajduch, Radu Pislariu, Juan Bautista De Sanctis, Danuta Radzioch
Front Pharmacol. 2022; 13: 876842. Published online 2022 May 20. doi: 10.3389/fphar.2022.876842
PMCID:
PMC9163687
AbstractArticlePDF–5.2M
19.
Alterations of mucosa-attached microbiome and epithelial cell numbers in the cystic fibrosis small intestine with implications for intestinal disease
Jennifer Kelly, Miran Al-Rammahi, Kristian Daly, Paul K. Flanagan, Arun Urs, Marta C. Cohen, Gabriella di Stefano, Marcel J. C. Bijvelds, David N. Sheppard, Hugo R. de Jonge, Ursula E. Seidler, Soraya P. Shirazi-Beechey
Sci Rep. 2022; 12: 6593. Published online 2022 Apr 21. doi: 10.1038/s41598-022-10328-3
PMCID:
PMC9023491
AbstractArticlePDF–12M
20.
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice
Mélanie Faria da Cunha, Iwona Pranke, Ali Sassi, Christiane Schreiweis, Stéphanie Moriceau, Dragana Vidovic, Aurélie Hatton, Mariane Sylvia Carlon, Geordie Creste, Farouk Berhal, Guillaume Prestat, Romain Freund, Norbert Odolczyk, Jean Philippe Jais, Christine Gravier-Pelletier, Piotr Zielenkiewicz, Vincent Jullien, Alexandre Hinzpeter, Franck Oury, Aleksander Edelman, Isabelle Sermet-Gaudelus
Sci Rep. 2022; 12: 6132. Published online 2022 Apr 12. doi: 10.1038/s41598-022-09678-9
PMCID:
PMC9005718
AbstractArticlePDF–1.5M

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