Literature DB >> 26406363

A combination therapy for cystic fibrosis.

Jeffrey L Brodsky1, Raymond A Frizzell2.   

Abstract

The most prevalent form of cystic fibrosis arises from an amino acid deletion in the cystic fibrosis transmembrane conductance regulator, CFTR. A recently approved treatment for individuals homozygous for this mutation combines a chemical corrector, which helps CFTR fold, and a potentiator that increases CFTR channel activity.
Copyright © 2015 Elsevier Inc. All rights reserved.

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Year:  2015        PMID: 26406363      PMCID: PMC4865247          DOI: 10.1016/j.cell.2015.09.003

Source DB:  PubMed          Journal:  Cell        ISSN: 0092-8674            Impact factor:   41.582


  7 in total

1.  Modulation of TMEM16A channel activity by the von Willebrand factor type A (VWA) domain of the calcium-activated chloride channel regulator 1 (CLCA1).

Authors:  Monica Sala-Rabanal; Zeynep Yurtsever; Kayla N Berry; Colin G Nichols; Tom J Brett
Journal:  J Biol Chem       Date:  2017-04-18       Impact factor: 5.157

2.  Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.

Authors:  Deborah M Cholon; Charles R Esther; Martina Gentzsch
Journal:  Expert Rev Precis Med Drug Dev       Date:  2016-04-22

Review 3.  Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications.

Authors:  Michelle L McClure; Stephen Barnes; Jeffrey L Brodsky; Eric J Sorscher
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2016-07-29       Impact factor: 5.464

4.  Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy.

Authors:  Romina Fiorotto; Mariangela Amenduni; Valeria Mariotti; Luca Fabris; Carlo Spirli; Mario Strazzabosco
Journal:  Hepatology       Date:  2018-01-26       Impact factor: 17.425

5.  S-Nitrosylation of CHIP Enhances F508Del-CFTR Maturation.

Authors:  Khalequz Zaman; Julia Knight; Faraaz Hussain; Ruofan Cao; Samuel K Estabrooks; Ghaith Altawallbeh; Kristopher Holloway; Anjum Jafri; Victoria Sawczak; Yuejin Li; Paulina Getsy; Fei Sun; Thomas Raffay; Calvin Cotton; Jeffrey L Brodsky; Ammasi Periasamy; Stephen J Lewis; Benjamin Gaston
Journal:  Am J Respir Cell Mol Biol       Date:  2019-12       Impact factor: 6.914

6.  Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.

Authors:  Vedrana Bali; Ahmed Lazrak; Purushotham Guroji; Sadis Matalon; Zsuzsanna Bebok
Journal:  PLoS One       Date:  2016-05-23       Impact factor: 3.240

7.  Identification of small-molecule ion channel modulators in C. elegans channelopathy models.

Authors:  Qiang Jiang; Kai Li; Wen-Jing Lu; Shuang Li; Xin Chen; Xi-Juan Liu; Jie Yuan; Qiurong Ding; Feng Lan; Shi-Qing Cai
Journal:  Nat Commun       Date:  2018-09-26       Impact factor: 14.919

  7 in total

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