 Cystic Fibrosis Transmembrane Conductance Regulator
"Cystic Fibrosis Transmembrane Conductance Regulator" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)
| Descriptor ID |
D019005
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| MeSH Number(s) |
D12.776.157.530.400.175.125 D12.776.543.550.425.175.125 D12.776.543.585.400.175.125
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| Concept/Terms |
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Below are MeSH descriptors whose meaning is more general than "Cystic Fibrosis Transmembrane Conductance Regulator".
Below are MeSH descriptors whose meaning is more specific than "Cystic Fibrosis Transmembrane Conductance Regulator".
This graph shows the total number of publications written about "Cystic Fibrosis Transmembrane Conductance Regulator" by people in UAMS Profiles by year, and whether "Cystic Fibrosis Transmembrane Conductance Regulator" was a major or minor topic of these publications.
To see the data from this visualization as text, click here.
| Year | Major Topic | Minor Topic | Total |
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| 2024 | 2 | 2 | 4 | | 2023 | 0 | 1 | 1 | | 2022 | 0 | 5 | 5 | | 2021 | 2 | 3 | 5 | | 2020 | 2 | 0 | 2 | | 2019 | 0 | 1 | 1 | | 2018 | 1 | 1 | 2 | | 2017 | 2 | 0 | 2 | | 2016 | 1 | 0 | 1 | | 2015 | 1 | 1 | 2 | | 2014 | 1 | 0 | 1 | | 2012 | 1 | 0 | 1 | | 2010 | 1 | 1 | 2 | | 2009 | 0 | 1 | 1 | | 2008 | 1 | 0 | 1 | | 2004 | 1 | 0 | 1 | | 2002 | 1 | 0 | 1 |
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Below are the most recent publications written about "Cystic Fibrosis Transmembrane Conductance Regulator" by people in Profiles over the past ten years.
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Rose NR, Dabbs SG, O'Hagan EC, Guimbellot JS. Literary evidence of the impact of nonbiological risk factors on CRMS/CFSPID: A scoping review. Pediatr Pulmonol. 2024 Dec; 59(12):3095-3105.
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Baker E, Harris WT, Guimbellot JS, Bliton K, Rowe SM, Raju SV, Oates GR. Association between biomarkers of tobacco smoke exposure and clinical efficacy of ivacaftor in the G551D observational trial (GOAL). J Cyst Fibros. 2024 Sep; 23(5):959-966.
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Liu Z, Anderson JD, Rose NR, Baker EH, Dowell AE, Ryan KJ, Acosta EP, Guimbellot JS. Differential distribution of ivacaftor and its metabolites in plasma and human airway epithelia. Pulm Pharmacol Ther. 2024 Sep; 86:102314.
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Rose NR, Chalamalla AR, Garcia BA, Krick S, Bergeron J, Sadeghi H, Schellhase DE, Ryan KJ, Dowell AE, Acosta EP, Guimbellot JS. Pharmacokinetic variability of CFTR modulators from standard and alternative regimens. Pulm Pharmacol Ther. 2024 Sep; 86:102301.
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Li C, Liu Z, Anderson J, Liu Z, Tang L, Li Y, Peng N, Chen J, Liu X, Fu L, Townes TM, Rowe SM, Bedwell DM, Guimbellot J, Zhao R. Prime editing-mediated correction of the CFTR W1282X mutation in iPSCs and derived airway epithelial cells. PLoS One. 2023; 18(11):e0295009.
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Gushue C, Eisner M, Bai S, Johnson T, Holtzlander M, McCoy K, Sheikh S. Impact of Elexacaftor-Tezacaftor-Ivacaftor on lung disease in cystic fibrosis. Pediatr Pulmonol. 2023 Aug; 58(8):2308-2316.
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Zhang S, Shrestha CL, Robledo-Avila F, Jaganathan D, Wisniewski BL, Brown N, Pham H, Carey K, Amer AO, Hall-Stoodley L, McCoy KS, Bai S, Partida-Sanchez S, Kopp BT. Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor. Eur Respir J. 2023 04; 61(4).
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McCoy KS, Blind J, Johnson T, Olson P, Raterman L, Bai S, Eisner M, Sheikh SI, Druhan S, Young C, Pasley K. Clinical change 2 years from start of elexacaftor-tezacaftor-ivacaftor in severe cystic fibrosis. Pediatr Pulmonol. 2023 04; 58(4):1178-1184.
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Guimbellot JS, Nichols DP, Brewington JJ. Novel Applications of Biomarkers and Personalized Medicine in Cystic Fibrosis. Clin Chest Med. 2022 12; 43(4):617-630.
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Mayer-Hamblett N, Ratjen F, Russell R, Donaldson SH, Riekert KA, Sawicki GS, Odem-Davis K, Young JK, Rosenbluth D, Taylor-Cousar JL, Goss CH, Retsch-Bogart G, Clancy JP, Genatossio A, O'Sullivan BP, Berlinski A, Millard SL, Omlor G, Wyatt CA, Moffett K, Nichols DP, Gifford AH. Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials. Lancet Respir Med. 2023 04; 11(4):329-340.
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Ramsey ML, Li SS, Lara LF, Gokun Y, Akshintala VS, Conwell DL, Heintz J, Kirkby SE, McCoy KS, Papachristou GI, Patel A, Singh VK, Hart PA. Cystic fibrosis transmembrane conductance regulator modulators and the exocrine pancreas: A scoping review. J Cyst Fibros. 2023 03; 22(2):193-200.
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Guimbellot JS, Ryan KJ, Anderson JD, Parker KL, Victoria Odom L, Rowe SM, Acosta EP. Plasma and cellular ivacaftor concentrations in patients with cystic fibrosis. Pediatr Pulmonol. 2022 11; 57(11):2745-2753.
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Ramos KJ, Guimbellot JS, Valapour M, Bartlett LE, Wai TH, Goss CH, Pilewski JM, Faro A, Diamond JM. Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients. J Cyst Fibros. 2022 09; 21(5):745-752.
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Liu Z, Anderson JD, Natt J, Guimbellot JS. Culture and Imaging of Human Nasal Epithelial Organoids. J Vis Exp. 2021 12 17; (178).
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Ramsey ML, Gokun Y, Sobotka LA, Wellner MR, Porter K, Kirkby SE, Li SS, Papachristou GI, Krishna SG, Stanich PP, Hart PA, Conwell DL, Lara LF. Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis. Am J Gastroenterol. 2021 12 01; 116(12):2446-2454.
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Anderson JD, Liu Z, Odom LV, Kersh L, Guimbellot JS. CFTR function and clinical response to modulators parallel nasal epithelial organoid swelling. Am J Physiol Lung Cell Mol Physiol. 2021 07 01; 321(1):L119-L129.
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Guimbellot JS, Taylor-Cousar JL. Combination CFTR modulator therapy in children and adults with cystic fibrosis. Lancet Respir Med. 2021 07; 9(7):677-679.
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Mainz JG, Arnold C, Wittstock K, Hipler UC, Lehmann T, Zagoya C, Duckstein F, Ellemunter H, Hentschel J. Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid From Cystic Fibrosis Patients With a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid. Front Immunol. 2021; 12:642180.
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Biesecker LG, Adam MP, Alkuraya FS, Amemiya AR, Bamshad MJ, Beck AE, Bennett JT, Bird LM, Carey JC, Chung B, Clark RD, Cox TC, Curry C, Dinulos MBP, Dobyns WB, Giampietro PF, Girisha KM, Glass IA, Graham JM, Gripp KW, Haldeman-Englert CR, Hall BD, Innes AM, Kalish JM, Keppler-Noreuil KM, Kosaki K, Kozel BA, Mirzaa GM, Mulvihill JJ, Nowaczyk MJM, Pagon RA, Retterer K, Rope AF, Sanchez-Lara PA, Seaver LH, Shieh JT, Slavotinek AM, Sobering AK, Stevens CA, Stevenson DA, Tan TY, Tan WH, Tsai AC, Weaver DD, Williams MS, Zackai E, Zarate YA. A dyadic approach to the delineation of diagnostic entities in clinical genomics. Am J Hum Genet. 2021 01 07; 108(1):8-15.
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Guimbellot JS, Baines A, Paynter A, Heltshe SL, VanDalfsen J, Jain M, Rowe SM, Sagel SD. Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation. J Cyst Fibros. 2021 03; 20(2):213-219.
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Huang J, Lam H, Koziol-White C, Limjunyawong N, Kim D, Kim N, Karmacharya N, Rajkumar P, Firer D, Dalesio NM, Jude J, Kurten RC, Pluznick JL, Deshpande DA, Penn RB, Liggett SB, Panettieri RA, Dong X, An SS. The odorant receptor OR2W3 on airway smooth muscle evokes bronchodilation via a cooperative chemosensory tradeoff between TMEM16A and CFTR. Proc Natl Acad Sci U S A. 2020 11 10; 117(45):28485-28495.
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Liu Z, Anderson JD, Deng L, Mackay S, Bailey J, Kersh L, Rowe SM, Guimbellot JS. Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis. Genes (Basel). 2020 05 29; 11(6).
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Zhang X, Pan A, Jia S, Ideozu JE, Woods K, Murkowski K, Hessner MJ, Simpson PM, Levy H. Cystic Fibrosis Plasma Blunts the Immune Response to Bacterial Infection. Am J Respir Cell Mol Biol. 2019 09; 61(3):301-311.
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Levy H, Jia S, Pan A, Zhang X, Kaldunski M, Nugent ML, Reske M, Feliciano RA, Quintero D, Renda MM, Woods KJ, Murkowski K, Johnson K, Verbsky J, Dasu T, Ideozu JE, McColley S, Quasney MW, Dahmer MK, Avner E, Farrell PM, Cannon CL, Jacob H, Simpson PM, Hessner MJ. Identification of molecular signatures of cystic fibrosis disease status with plasma-based functional genomics. Physiol Genomics. 2019 01 01; 51(1):27-41.
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Guimbellot J, Solomon GM, Baines A, Heltshe SL, VanDalfsen J, Joseloff E, Sagel SD, Rowe SM. Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations. J Cyst Fibros. 2019 01; 18(1):102-109.
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Guimbellot JS, Leach JM, Chaudhry IG, Quinney NL, Boyles SE, Chua M, Aban I, Jaspers I, Gentzsch M. Nasospheroids permit measurements of CFTR-dependent fluid transport. JCI Insight. 2017 11 16; 2(22).
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Guimbellot J, Sharma J, Rowe SM. Toward inclusive therapy with CFTR modulators: Progress and challenges. Pediatr Pulmonol. 2017 Nov; 52(S48):S4-S14.
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Perez-Marques F, Simpson P, Yan K, Quasney MW, Halligan N, Merchant D, Dahmer MK. Association of polymorphisms in genes of factors involved in regulation of splicing of cystic fibrosis transmembrane conductance regulator mRNA with acute respiratory distress syndrome in children with pneumonia. Crit Care. 2016 09 05; 20:281.
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Illing EA, Cho DY, Zhang S, Skinner DF, Dunlap QA, Sorscher EJ, Woodworth BA. Chlorogenic Acid Activates CFTR-Mediated Cl- Secretion in Mice and Humans: Therapeutic Implications for Chronic Rhinosinusitis. Otolaryngol Head Neck Surg. 2015 Aug; 153(2):291-7.
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Grant CN, Mojica SG, Sala FG, Hill JR, Levin DE, Speer AL, Barthel ER, Shimada H, Zachos NC, Grikscheit TC. Human and mouse tissue-engineered small intestine both demonstrate digestive and absorptive function. Am J Physiol Gastrointest Liver Physiol. 2015 Apr 15; 308(8):G664-77.
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