Ivacaftor (BioDeep_00000858459)
代谢物信息卡片
Ivacaftor (VX-770)
化学式: C24H28N2O3 (392.20998180000004)
中文名称: 依伐卡托
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CC(C)(C)C1=CC(=C(C=C1NC(=O)C2=CNC3=CC=CC=C3C2=O)O)C(C)(C)C
InChI: InChI=1S/C24H28N2O3/c1-23(2,3)16-11-17(24(4,5)6)20(27)12-19(16)26-22(29)15-13-25-18-10-8-7-9-14(18)21(15)28/h7-13,27H,1-6H3,(H,25,28)(H,26,29)
描述信息
D049990 - Membrane Transport Modulators > D065101 - Chloride Channel Agonists
C87006 - Pharmacological Chaperone
R - Respiratory system
同义名列表
2 个代谢物同义名
数据库引用编号
8 个数据库交叉引用编号
- ChEBI: CHEBI:66901
- KEGGdrug: D09916
- PubChem: 16220172
- DrugBank: DB08820
- ChEMBL: CHEMBL2010601
- CAS: 1413431-05-6
- CAS: 1134822-00-6
- CAS: 873054-44-5
分类词条
相关代谢途径
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
0 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Kevin Lonabaugh, Galvin Li, Rhonda List, Reyna Huang, Amber James, Andrew Barros, Lindsay Somerville, Dana Albon. Real world study on elexacaftor-tezacaftor-ivacaftor impact on cholesterol levels in adults with cystic fibrosis.
Pharmacotherapy.
2024 Mar; 44(3):231-240. doi:
10.1002/phar.2903. [PMID: 38143243] - Tanvi Patel, Kimberly McBennett, Senthilkumar Sankararaman, Teresa Schindler, Krithika Sundaram, Nori Mercuri Minich, Sindhoosha Malay, Katherine Kutney. Impact of elexacaftor/tezacaftor/ivacaftor on lipid and fat-soluble vitamin levels and association with body mass index.
Pediatric pulmonology.
2024 Mar; 59(3):734-742. doi:
10.1002/ppul.26823. [PMID: 38179878] - Katherine A Despotes, Agathe S Ceppe, Scott H Donaldson. Alterations in lipids after initiation of highly effective modulators in people with cystic fibrosis.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2023 Nov; 22(6):1024-1026. doi:
10.1016/j.jcf.2023.10.002. [PMID: 37838486] - Parniya Arooj, David V Morrissy, Yvonne McCarthy, Tamara Vagg, Mairead McCarthy, Claire Fleming, Mary Daly, Joseph A Eustace, Desmond M Murphy, B J Plant. ROCK STUDY in CF: sustained anti-inflammatory effects of lumacaftor-ivacaftor in sputum and peripheral blood samples of adult patients with cystic fibrosis-an observational study.
BMJ open respiratory research.
2023 May; 10(1):. doi:
10.1136/bmjresp-2022-001590. [PMID: 37130650] - Jordana E Hoppe, Brandie D Wagner, J Kirk Harris, Steven M Rowe, Sonya L Heltshe, Emily M DeBoer, Scott D Sagel. Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2022 11; 21(6):950-958. doi:
10.1016/j.jcf.2022.03.012. [PMID: 35440409] - N J Ronan, G G Einarsson, J Deane, F Fouhy, M Rea, C Hill, F Shanahan, J S Elborn, R P Ross, M McCarthy, D M Murphy, J A Eustace, Tunney Mm, C Stanton, B J Plant. Modulation, microbiota and inflammation in the adult CF gut: A prospective study.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2022 09; 21(5):837-843. doi:
10.1016/j.jcf.2022.06.002. [PMID: 35764510] - Eunjin Hong, Lisa M Almond, Peter S Chung, Adupa P Rao, Paul M Beringer. Physiologically-Based Pharmacokinetic-Led Guidance for Patients With Cystic Fibrosis Taking Elexacaftor-Tezacaftor-Ivacaftor With Nirmatrelvir-Ritonavir for the Treatment of COVID-19.
Clinical pharmacology and therapeutics.
2022 06; 111(6):1324-1333. doi:
10.1002/cpt.2585. [PMID: 35292968] - David Drummond, Jérémy Dana, Laureline Berteloot, Elena K Schneider-Futschik, Frédérique Chedevergne, Céline Bailly-Botuha, Thao Nguyen-Khoa, Mathieu Cornet, Muriel Le Bourgeois, Dominique Debray, Muriel Girard, Isabelle Sermet-Gaudelus. Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2022 03; 21(2):212-219. doi:
10.1016/j.jcf.2021.07.018. [PMID: 34454846] - Brittany A Wright, Natalie K Ketchen, Leah N Rasmussen, Alyssa R Bartels, Sachinkumar B Singh. Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients.
Pediatric pulmonology.
2022 03; 57(3):655-657. doi:
10.1002/ppul.25781. [PMID: 34859619] - Max C Petersen, Lauren Begnel, Michael Wallendorf, Marina Litvin. Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2022 03; 21(2):265-271. doi:
10.1016/j.jcf.2021.11.012. [PMID: 34862121] - Katharina Habler, Anne-Sophie Kalla, Michael Rychlik, Mathias Bruegel, Daniel Teupser, Susanne Nährig, Michael Vogeser, Michael Paal. Isotope dilution LC-MS/MS quantification of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators ivacaftor, lumacaftor, tezacaftor, elexacaftor, and their major metabolites in human serum.
Clinical chemistry and laboratory medicine.
2022 01; 60(1):82-91. doi:
10.1515/cclm-2021-0724. [PMID: 34668357] - Yanfei Miao, Shihua Zhao, Jian Zuo, Jiqin Sun, Jingnan Wang. Reduced the Food Effect and Enhanced the Oral Bioavailability of Ivacaftor by Self-Nanoemulsifying Drug Delivery System (SNEDDS) Using a New Oil Phase.
Drug design, development and therapy.
2022; 16(?):1531-1546. doi:
10.2147/dddt.s356967. [PMID: 35637746] - C Colombo, A Foppiani, A Bisogno, S Gambazza, V Daccò, E Nazzari, A Leone, A Giana, A Mari, A Battezzati. Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion.
Journal of endocrinological investigation.
2021 Oct; 44(10):2213-2218. doi:
10.1007/s40618-021-01525-4. [PMID: 33586024] - C E Pope, A T Vo, H S Hayden, E J Weiss, S Durfey, S McNamara, A Ratjen, B Grogan, S Carter, L Nay, M R Parsek, P K Singh, E F McKone, M L Aitken, M R Rosenfeld, L R Hoffman. Changes in fecal microbiota with CFTR modulator therapy: A pilot study.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2021 09; 20(5):742-746. doi:
10.1016/j.jcf.2020.12.002. [PMID: 33390317] - Fiona Qiu, Mark D Habgood, Yifan Huang, Katarzyna M Dziegielewska, Sam Toll, Elena K Schneider-Futschik. Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2021 09; 20(5):857-864. doi:
10.1016/j.jcf.2021.06.005. [PMID: 34193363] - Vinciane Saint-Criq, Yiting Wang, Livia Delpiano, JinHeng Lin, David N Sheppard, Michael A Gray. Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2021 09; 20(5):843-850. doi:
10.1016/j.jcf.2021.04.013. [PMID: 34020896] - Joshena Jeyaratnam, Renske van der Meer, Gitte Berkers, Harry Gm Heijerman, Jeffrey M Beekman, Cornelis K van der Ent. Breast development in a 7 year old girl with CF treated with ivacaftor: An indication for personalized dosing?.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2021 09; 20(5):e63-e66. doi:
10.1016/j.jcf.2021.06.001. [PMID: 34175243] - Gisli G Einarsson, Nicola J Ronan, Denver Mooney, Clodagh McGettigan, David Mullane, Muireann NiChroinin, Fergus Shanahan, Desmond M Murphy, Mairead McCarthy, Yvonne McCarthy, Joseph A Eustace, Deirdre F Gilpin, J Stuart Elborn, Barry J Plant, Michael M Tunney. Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2021 09; 20(5):747-753. doi:
10.1016/j.jcf.2020.12.023. [PMID: 33549519] - Steffie E M Vonk, Marloes van der Meer-Vos, Lieuwe D J Bos, Anne H Neerincx, Christof J Majoor, Anke-Hilse Maitland-van der Zee, Ron A A Mathôt, E Marleen Kemper. Quantitative Method for the Analysis of Ivacaftor, Hydroxymethyl Ivacaftor, Ivacaftor Carboxylate, Lumacaftor, and Tezacaftor in Plasma and Sputum Using Liquid Chromatography With Tandem Mass Spectrometry and Its Clinical Applicability.
Therapeutic drug monitoring.
2021 08; 43(4):555-563. doi:
10.1097/ftd.0000000000000829. [PMID: 33165217] - Emma De Jong, Luke W Garratt, Kevin Looi, Amy H Y Lee, Kak-Ming Ling, Maren L Smith, Reza Falsafi, Erika N Sutanto, Jessica Hillas, Thomas Iosifidis, Kelly M Martinovich, Nicole C Shaw, Samuel T Montgomery, Elizabeth Kicic-Starcevich, Francis J Lannigan, Shyan Vijayasekaran, Robert E W Hancock, Stephen M Stick, Anthony Kicic, C F Arest. Ivacaftor or lumacaftor/ivacaftor treatment does not alter the core CF airway epithelial gene response to rhinovirus.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2021 01; 20(1):97-105. doi:
10.1016/j.jcf.2020.07.004. [PMID: 32684439] - Stella Prins, Emily Langron, Cato Hastings, Emily J Hill, Andra C Stefan, Lewis D Griffin, Paola Vergani. Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
The Journal of biological chemistry.
2020 12; 295(49):16529-16544. doi:
10.1074/jbc.ra120.014061. [PMID: 32934006] - Gitte Berkers, Renske van der Meer, Peter van Mourik, Annelotte M Vonk, Evelien Kruisselbrink, Sylvia Wf Suen, Harry Gm Heijerman, Christof J Majoor, Gerard H Koppelman, Jolt Roukema, Hettie M Janssens, Yolanda B de Rijke, E Marleen Kemper, Jeffrey M Beekman, Cornelis K van der Ent, Hugo R de Jonge. Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2020 11; 19(6):955-961. doi:
10.1016/j.jcf.2020.04.014. [PMID: 32499204] - Chune Zhu, Jianting Chen, Shihui Yu, Chailu Que, Lynne S Taylor, Wen Tan, Chuanbin Wu, Qi Tony Zhou. Inhalable Nanocomposite Microparticles with Enhanced Dissolution and Superior Aerosol Performance.
Molecular pharmaceutics.
2020 09; 17(9):3270-3280. doi:
10.1021/acs.molpharmaceut.0c00390. [PMID: 32643939] - A L Nichols, J C Davies, D Jones, S B Carr. Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor.
Paediatric respiratory reviews.
2020 Sep; 35(?):99-102. doi:
10.1016/j.prrv.2020.04.003. [PMID: 32386958] - Tara N Guhr Lee, Deborah M Cholon, Nancy L Quinney, Martina Gentzsch, Charles R Esther. Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2020 09; 19(5):746-751. doi:
10.1016/j.jcf.2020.04.010. [PMID: 32536510] - Jennifer S Guimbellot, Kevin J Ryan, Justin D Anderson, Zhongyu Liu, Latona Kersh, Charles R Esther, Steven M Rowe, Edward P Acosta. Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2020 09; 19(5):742-745. doi:
10.1016/j.jcf.2020.01.011. [PMID: 32044246] - Nara Liessi, Emanuela Pesce, Clarissa Braccia, Sine Mandrup Bertozzi, Alessandro Giraudo, Tiziano Bandiera, Nicoletta Pedemonte, Andrea Armirotti. Distinctive lipid signatures of bronchial epithelial cells associated with cystic fibrosis drugs, including Trikafta.
JCI insight.
2020 08; 5(16):. doi:
10.1172/jci.insight.138722. [PMID: 32673287] - Elodie Mareux, Martine Lapalus, Rachida Amzal, Marion Almes, Tounsia Aït-Slimane, Jean-Louis Delaunay, Pauline Adnot, Mauricette Collado-Hilly, Anne Davit-Spraul, Thomas Falguières, Isabelle Callebaut, Emmanuel Gonzales, Emmanuel Jacquemin. Functional rescue of an ABCB11 mutant by ivacaftor: A new targeted pharmacotherapy approach in bile salt export pump deficiency.
Liver international : official journal of the International Association for the Study of the Liver.
2020 08; 40(8):1917-1925. doi:
10.1111/liv.14518. [PMID: 32433800] - Fanny Degrugillier, Abdel Aissat, Virginie Prulière-Escabasse, Lucie Bizard, Benjamin Simonneau, Xavier Decrouy, Chong Jiang, Daniela Rotin, Pascale Fanen, Stéphanie Simon. Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.
International journal of molecular sciences.
2020 Jul; 21(14):. doi:
10.3390/ijms21144844. [PMID: 32650630] - Inna Uliyakina, Hugo M Botelho, Ana C da Paula, Sara Afonso, Miguel J Lobo, Verónica Felício, Carlos M Farinha, Margarida D Amaral. Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions.
International journal of molecular sciences.
2020 Jun; 21(12):. doi:
10.3390/ijms21124524. [PMID: 32630527] - Giulia Mancini, Nicoletta Loberto, Debora Olioso, Maria Cristina Dechecchi, Giulio Cabrini, Laura Mauri, Rosaria Bassi, Domitilla Schiumarini, Elena Chiricozzi, Giuseppe Lippi, Emanuela Pesce, Sandro Sonnino, Nicoletta Pedemonte, Anna Tamanini, Massimo Aureli. GM1 as Adjuvant of Innovative Therapies for Cystic Fibrosis Disease.
International journal of molecular sciences.
2020 Jun; 21(12):. doi:
10.3390/ijms21124486. [PMID: 32599772] - Kirsten A Cottrill, Carlos M Farinha, Nael A McCarty. The bidirectional relationship between CFTR and lipids.
Communications biology.
2020 04; 3(1):179. doi:
10.1038/s42003-020-0909-1. [PMID: 32313074] - Colm Kerr, David Morrissy, Mary Horgan, Barry J Plant. Microbial clues lead to a diagnosis of cystic fibrosis in late adulthood.
BMJ case reports.
2020 Apr; 13(4):. doi:
10.1136/bcr-2019-233470. [PMID: 32317365] - Heledd H Jarosz-Griffiths, Thomas Scambler, Chi H Wong, Samuel Lara-Reyna, Jonathan Holbrook, Fabio Martinon, Sinisa Savic, Paul Whitaker, Christine Etherington, Giulia Spoletini, Ian Clifton, Anil Mehta, Michael F McDermott, Daniel Peckham. Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis.
eLife.
2020 03; 9(?):. doi:
10.7554/elife.54556. [PMID: 32118580] - Guido Veit, Dillon F Da Fonte, Radu G Avramescu, Aiswarya Premchandar, Miklos Bagdany, Haijin Xu, Dennis Bensinger, Daniel Stubba, Boris Schmidt, Elias Matouk, Gergely L Lukacs. Mutation-specific dual potentiators maximize rescue of CFTR gating mutants.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2020 03; 19(2):236-244. doi:
10.1016/j.jcf.2019.10.011. [PMID: 31678009] - Scott C Bell, Peter J Barry, Kris De Boeck, Pavel Drevinek, J Stuart Elborn, Barry J Plant, Predag Minić, Eva Van Braeckel, Stijn Verhulst, Karine Muller, Desirée Kanters, Susan Bellaire, Herman de Kock, David E Geller, Katja Conrath, Olivier Van de Steen, Kors van der Ent. CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2019 09; 18(5):700-707. doi:
10.1016/j.jcf.2019.04.014. [PMID: 31056441] - Susanna Kinting, Yang Li, Maria Forstner, Florent Delhommel, Michael Sattler, Matthias Griese. Potentiation of ABCA3 lipid transport function by ivacaftor and genistein.
Journal of cellular and molecular medicine.
2019 08; 23(8):5225-5234. doi:
10.1111/jcmm.14397. [PMID: 31210424] - Samuel J Bose, Marcel J C Bijvelds, Yiting Wang, Jia Liu, Zhiwei Cai, Alice G M Bot, Hugo R de Jonge, David N Sheppard. Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.
American journal of physiology. Lung cellular and molecular physiology.
2019 07; 317(1):L71-L86. doi:
10.1152/ajplung.00034.2019. [PMID: 30969810] - John J McNamara, Susanna A McColley, Gautham Marigowda, Fang Liu, Simon Tian, Caroline A Owen, David Stiles, Chonghua Li, David Waltz, Linda T Wang, Gregory S Sawicki. Safety, pharmacokinetics, and pharmacodynamics of lumacaftor and ivacaftor combination therapy in children aged 2-5 years with cystic fibrosis homozygous for F508del-CFTR: an open-label phase 3 study.
The Lancet. Respiratory medicine.
2019 04; 7(4):325-335. doi:
10.1016/s2213-2600(18)30460-0. [PMID: 30686767] - Béla Nagy, Zsolt Bene, Zsolt Fejes, Sonya L Heltshe, David Reid, Nicola J Ronan, Yvonne McCarthy, Daniel Smith, Attila Nagy, Elizabeth Joseloff, György Balla, János Kappelmayer, Milan Macek, Scott C Bell, Barry J Plant, Margarida D Amaral, István Balogh. Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV1) in cystic fibrosis patients receiving ivacaftor treatment.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2019 03; 18(2):271-277. doi:
10.1016/j.jcf.2018.08.013. [PMID: 30268371] - Ivo P van de Peppel, Marcela Doktorova, Gitte Berkers, Hugo R de Jonge, Roderick H J Houwen, Henkjan J Verkade, Johan W Jonker, Frank A J A Bodewes. IVACAFTOR restores FGF19 regulated bile acid homeostasis in cystic fibrosis patients with an S1251N or a G551D gating mutation.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.
2019 03; 18(2):286-293. doi:
10.1016/j.jcf.2018.09.001. [PMID: 30279125] - O B Garbuzenko, N Kbah, A Kuzmov, N Pogrebnyak, V Pozharov, T Minko. Inhalation treatment of cystic fibrosis with lumacaftor and ivacaftor co-delivered by nanostructured lipid carriers.
Journal of controlled release : official journal of the Controlled Release Society.
2019 02; 296(?):225-231. doi:
10.1016/j.jconrel.2019.01.025. [PMID: 30677435] - Laura Kirwan, Godfrey Fletcher, Mary Harrington, Paulina Jeleniewska, Shijun Zhou, Brian Casserly, Charles G Gallagher, Peter Greally, Cedric Gunaratnam, Mary Herzig, Barry Linnane, Noel Gerard McElvaney, Edward F McKone, Paul McNally, David Mullane, Muireann Ní Chróinín, Michael O'Mahony, Barry J Plant, Abaigeal D Jackson. Longitudinal Trends in Real-World Outcomes after Initiation of Ivacaftor. A Cohort Study from the Cystic Fibrosis Registry of Ireland.
Annals of the American Thoracic Society.
2019 02; 16(2):209-216. doi:
10.1513/annalsats.201802-149oc. [PMID: 30427731] - Jane C Davies, Samuel M Moskowitz, Cynthia Brown, Alexander Horsley, Marcus A Mall, Edward F McKone, Barry J Plant, Dario Prais, Bonnie W Ramsey, Jennifer L Taylor-Cousar, Elizabeth Tullis, Ahmet Uluer, Charlotte M McKee, Sarah Robertson, Rebecca A Shilling, Christopher Simard, Fredrick Van Goor, David Waltz, Fengjuan Xuan, Tim Young, Steven M Rowe. VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
The New England journal of medicine.
2018 10; 379(17):1599-1611. doi:
10.1056/nejmoa1807119. [PMID: 30334693] - Frédéric Velard, Marie Laure Jourdain, Dina Abdallah, Jacky Jacquot, Nicola Ronan, Yvonne McCarthy, Evelyn Flanagan, Barry Plant. Overexpression of RANK and M-CSFR in Monocytes of G551D-Bearing Patients with Cystic Fibrosis.
American journal of respiratory and critical care medicine.
2018 10; 198(7):968-970. doi:
10.1164/rccm.201803-0415le. [PMID: 29940131] - Ana M Matos, Andreia Gomes-Duarte, Márcia Faria, Patrícia Barros, Peter Jordan, Margarida D Amaral, Paulo Matos. Prolonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR.
Scientific reports.
2018 08; 8(1):13026. doi:
10.1038/s41598-018-31514-2. [PMID: 30158635] - Stephanie Chin, Maurita Hung, Amy Won, Yu-Sheng Wu, Saumel Ahmadi, Donghe Yang, Salma Elmallah, Krimo Toutah, C Michael Hamilton, Robert N Young, Russell D Viirre, Christopher M Yip, Christine E Bear. Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.
Molecular pharmacology.
2018 08; 94(2):917-925. doi:
10.1124/mol.118.112177. [PMID: 29903751] - Nicola J Ronan, Gisli G Einarsson, Maria Twomey, Denver Mooney, David Mullane, Muireann NiChroinin, Grace O'Callaghan, Fergus Shanahan, Desmond M Murphy, Owen J O'Connor, Cathy A Shortt, Michael M Tunney, Joseph A Eustace, Michael M Maher, J Stuart Elborn, Barry J Plant. CORK Study in Cystic Fibrosis: Sustained Improvements in Ultra-Low-Dose Chest CT Scores After CFTR Modulation With Ivacaftor.
Chest.
2018 02; 153(2):395-403. doi:
10.1016/j.chest.2017.10.005. [PMID: 29037527] - Nara Liessi, Elena Cichero, Emanuela Pesce, Maria Arkel, Annalisa Salis, Valeria Tomati, Matteo Paccagnella, Gianluca Damonte, Bruno Tasso, Luis J V Galietta, Nicoletta Pedemonte, Paola Fossa, Enrico Millo. Synthesis and biological evaluation of novel thiazole- VX-809 hybrid derivatives as F508del correctors by QSAR-based filtering tools.
European journal of medicinal chemistry.
2018 Jan; 144(?):179-200. doi:
10.1016/j.ejmech.2017.12.030. [PMID: 29272749] - Anne Marie Liddy, Gavin McLaughlin, Susanne Schmitz, Deirdre M D'Arcy, Michael G Barry. The pharmacokinetic interaction between ivacaftor and ritonavir in healthy volunteers.
British journal of clinical pharmacology.
2017 Oct; 83(10):2235-2241. doi:
10.1111/bcp.13324. [PMID: 28477428] - Emily Langron, Michela I Simone, Clémence M S Delalande, Jean-Louis Reymond, David L Selwood, Paola Vergani. Improved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds.
British journal of pharmacology.
2017 04; 174(7):525-539. doi:
10.1111/bph.13715. [PMID: 28094839] - Xin Meng, Yiting Wang, Xiaomeng Wang, Joe A Wrennall, Tracy L Rimington, Hongyu Li, Zhiwei Cai, Robert C Ford, David N Sheppard. Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
The Journal of biological chemistry.
2017 03; 292(9):3706-3719. doi:
10.1074/jbc.m116.751537. [PMID: 28087700] - Jean-Louis Delaunay, Alix Bruneau, Brice Hoffmann, Anne-Marie Durand-Schneider, Véronique Barbu, Emmanuel Jacquemin, Michèle Maurice, Chantal Housset, Isabelle Callebaut, Tounsia Aït-Slimane. Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770).
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