Zobrazeno 1 - 10
of 45
pro vyhledávání: '"Guido Veit"'
Publikováno v:
Frontiers in Pharmacology, Vol 15 (2024)
Externí odkaz:
https://doaj.org/article/7d9167ebf42f4eb493a4889e34ee602c
Autor:
Aiswarya Premchandar, Ruiji Ming, Abed Baiad, Dillon F. Da Fonte, Haijin Xu, Denis Faubert, Guido Veit, Gergely L. Lukacs
Publikováno v:
Frontiers in Pharmacology, Vol 15 (2024)
Cystic fibrosis (CF) is a monogenic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. Premature termination codons (PTCs) represent ∼9% of CF mutations that typically cause severe expression defects of the CFTR
Externí odkaz:
https://doaj.org/article/0054f0d55fa143fcb730664fc66df904
Autor:
Naoto Soya, Haijin Xu, Ariel Roldan, Zhengrong Yang, Haoxin Ye, Fan Jiang, Aiswarya Premchandar, Guido Veit, Susan P. C. Cole, John Kappes, Tamás Hegedüs, Gergely L. Lukacs
Publikováno v:
Nature Communications, Vol 14, Iss 1, Pp 1-21 (2023)
Abstract The folding/misfolding and pharmacological rescue of multidomain ATP-binding cassette (ABC) C-subfamily transporters, essential for organismal health, remain incompletely understood. The ABCC transporters core consists of two nucleotide bind
Externí odkaz:
https://doaj.org/article/26ee4a0b5ae048ff8a59c8ecf53a2afc
Publikováno v:
Frontiers in Molecular Biosciences, Vol 9 (2022)
Externí odkaz:
https://doaj.org/article/a97aa94a9e1c45a7854c2c0302a77cae
Autor:
Guido Veit, Ariel Roldan, Mark A. Hancock, Dillon F. Da Fonte, Haijin Xu, Maytham Hussein, Saul Frenkiel, Elias Matouk, Tony Velkov, Gergely L. Lukacs
Publikováno v:
JCI Insight, Vol 5, Iss 18 (2020)
Based on its clinical benefits, Trikafta — the combination of folding correctors VX-661 (tezacaftor), VX-445 (elexacaftor), and the gating potentiator VX-770 (ivacaftor) — was FDA approved for treatment of patients with cystic fibrosis (CF) carry
Externí odkaz:
https://doaj.org/article/b1202180e975466c997feaa921fed85e
Autor:
Miklos Bagdany, Guido Veit, Ryosuke Fukuda, Radu G. Avramescu, Tsukasa Okiyoneda, Imad Baaklini, Jay Singh, Guy Sovak, Haijin Xu, Pirjo M. Apaja, Sara Sattin, Lenore K. Beitel, Ariel Roldan, Giorgio Colombo, William Balch, Jason C. Young, Gergely L. Lukacs
Publikováno v:
Nature Communications, Vol 8, Iss 1, Pp 1-15 (2017)
The F508 deletion (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) is the most common CF causing mutation. Here the authors show that cytosolic chaperones shift the F508del channel conformation to the native fold by kinetic
Externí odkaz:
https://doaj.org/article/e14a35b0276c400f88eedbb10dd40d9d
Autor:
Guido Veit, Kathryn Oliver, Pirjo M Apaja, Doranda Perdomo, Aurélien Bidaud-Meynard, Sheng-Ting Lin, Jingyu Guo, Mert Icyuz, Eric J Sorscher, John L Hartman, Gergely L Lukacs
Publikováno v:
PLoS Biology, Vol 14, Iss 5, p e1002462 (2016)
The most common cystic fibrosis (CF) causing mutation, deletion of phenylalanine 508 (ΔF508 or Phe508del), results in functional expression defect of the CF transmembrane conductance regulator (CFTR) at the apical plasma membrane (PM) of secretory e
Externí odkaz:
https://doaj.org/article/2e5a3f66228046208e973fd23ba3f520
Publikováno v:
Mediators of Inflammation, Vol 2016 (2016)
The pivotal role of epithelial cells is to secrete and absorb ions and water in order to allow the formation of a luminal fluid compartment that is fundamental for the epithelial function as a barrier against environmental factors. Importantly, epith
Externí odkaz:
https://doaj.org/article/a1339c6f7a504992bef4cb24d7e64976
Publikováno v:
J Cyst Fibros
Trikafta, the combination of elexacaftor (VX-445), tezacaftor (VX-661) and ivacaftor (VX-770), was approved for therapy of cystic fibrosis (CF) patients with at least one allele of the CFTR mutation F508del. While the corrector function of VX-445 is
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::147e1acee48ccdc48d1943b192a6efc7
https://doi.org/10.1016/j.jcf.2021.03.011
https://doi.org/10.1016/j.jcf.2021.03.011
Autor:
Chi Wang, Zhengrong Yang, Blaine J. Loughlin, Haijin Xu, Guido Veit, Sergey Vorobiev, Oliver B. Clarke, Fan Jiang, Yaohui Li, Shikha Singh, Zachary Rich, Elizabeth R. Menten, Robert A. Grassucci, Wei Wang, Allison Mezzell, Ziao Fu, Kam-Ho Wong, Jing Wang, Diana R. Wetmore, R. Bryan Sutton, Christie G. Brouillette, Ina L. Urbatsch, John C. Kappes, Gergely L. Lukacs, Joachim Frank, John F. Hunt
Cystic fibrosis (CF) is caused by mutations in a chloride channel called the human Cystic Fibrosis Transmembrane Conductance Regulator (hCFTR). We used cryo-EM global conformational ensemble reconstruction to characterize the mechanism by which the b
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::d8099769d8252d91e0952af46cc5ab09
https://doi.org/10.1101/2022.10.10.510913
https://doi.org/10.1101/2022.10.10.510913
