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Select item 113012171.

Impaired intestinal free fatty acid transport followed by chylomicron malformation, not pancreatic insufficiency, cause metabolic defects in cystic fibrosis

Lihong Teng, Nikolaos Dedousis, Aneseh Adeshirlarijaney, Jitendra S. Kanshana, Min Liu, Craig A. Hodges, Alison B. Kohan

J Lipid Res. 2024 Jul; 65(7): 100551. Published online 2024 Jul 13. doi: 10.1016/j.jlr.2024.100551

PMCID:: PMC11301217

Abstract Article PDF–1.5M

Select item 112713792.

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

Abstract Article PDF–5.6M

Select item 109301793.

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

Abstract Article PDF–1.6M

Select item 106539274.

The cystic fibrosis treatment Trikafta affects the growth, viability, and cell wall of Aspergillus fumigatus biofilms

Jane T. Jones, Kaesi A. Morelli, Elisa M. Vesely, Charles T. S. Puerner, Chetan K. Pavuluri, Brandon S. Ross, Norman van Rhijn, Michael J. Bromley, Robert A. Cramer

mBio. 2023 Sep-Oct; 14(5): e01516-23. Published online 2023 Oct 13. doi: 10.1128/mbio.01516-23

PMCID:: PMC10653927

Abstract Article PDF–2.3M

Select item 106405635.

Lung SORT LNPs enable precise homology-directed repair mediated CRISPR/Cas genome correction in cystic fibrosis models

Tuo Wei, Yehui Sun, Qiang Cheng, Sumanta Chatterjee, Zachary Traylor, Lindsay T. Johnson, Melissa L. Coquelin, Jialu Wang, Michael J. Torres, Xizhen Lian, Xu Wang, Yufen Xiao, Craig A. Hodges, Daniel J. Siegwart

Nat Commun. 2023; 14: 7322. Published online 2023 Nov 11. doi: 10.1038/s41467-023-42948-2

PMCID:: PMC10640563

Abstract Article PDF–2.0M

Select item 106356466.

CFTR and PC2, partners in the primary cilia in autosomal dominant polycystic kidney disease

Murali K. Yanda, Cristian Ciobanu, William B. Guggino, Liudmila Cebotaru

Am J Physiol Cell Physiol. 2023 Sep 1; 325(3): C682–C693. Published online 2023 Jul 31. doi: 10.1152/ajpcell.00197.2023

PMCID:: PMC10635646

Abstract Article

Select item 105798097.

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

Abstract Article PDF–3.3M

Select item 105772788.

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

Abstract Article PDF–1.9M

Select item 105757289.

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

Abstract Article PDF–4.7M

Select item 1045443810.

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

Abstract Article PDF–2.5M

Select item 1041637811.

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

Abstract Article PDF–2.9M

Select item 1039399612.

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

Abstract Article PDF–11M

Select item 1035340013.

Identification of Virulence Factors Involved in a Murine Model of Severe Achromobacter xylosoxidans Infection

Brandon M. Wills, Preeti Garai, Molly O. Riegert, Felix T. Sanchez, Adam M. Pickrum, Dara W. Frank, Kenneth L. Brockman

Infect Immun. 2023 Jul; 91(7): e00037-23. Published online 2023 May 31. doi: 10.1128/iai.00037-23

PMCID:: PMC10353400

Abstract Article PDF–2.8M

Select item 1033280514.

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

Abstract Article PDF–1.5M

Select item 1013900015.

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

Abstract Article PDF–2.9M

Select item 993397016.

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

Abstract Article PDF–4.9M

Select item 992511717.

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

Abstract Article

Select item 983383018.

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

Abstract Article PDF–1.7M

Select item 974319019.

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

Article PDF–686K

Select item 949003320.

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

Abstract Article PDF–3.3M

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