Zobrazeno 1 - 10
of 23
pro vyhledávání: '"Clément Boinot"'
Autor:
Emily Anne Smith Bergbower, Inna Sabirzhanova, Clément Boinot, William B. Guggino, Liudmila Cebotaru
Publikováno v:
Cellular Physiology and Biochemistry, Vol 52, Iss 6, Pp 1267-1279 (2019)
Externí odkaz:
https://doaj.org/article/6ad549979b1249a096b4e70ac778cad6
Publikováno v:
Cellular Physiology and Biochemistry, Vol 51, Iss 3, Pp 1489-1499 (2018)
Background/Aims: Cystic fibrosis (CF) is a lethal recessive disorder caused by mutations in the CF transmembrane conductance regulator (CFTR). ΔF508, the most common mutation, is a misfolded protein that is retained in the endoplasmic reticulum and
Externí odkaz:
https://doaj.org/article/72a194b27d754f52b3001997eeaefa36
Publikováno v:
Cellular Physiology and Biochemistry, Vol 41, Iss 6, Pp 2194-2210 (2017)
Background/Aims: Premature degradation of mutated cystic fibrosis transmembrane conductance regulator (CFTR) protein causes cystic fibrosis (CF), the commonest Mendelian disease in Caucasians. Despite recent advances in precision medicines for CF pat
Externí odkaz:
https://doaj.org/article/d1bd1adde0284c35a122fc0d9f0b6a7e
Publikováno v:
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology
Cellular Physiology and Biochemistry, Vol 51, Iss 3, Pp 1489-1499 (2018)
Cellular Physiology and Biochemistry, Vol 51, Iss 3, Pp 1489-1499 (2018)
Background/Aims: Cystic fibrosis (CF) is a lethal recessive disorder caused by mutations in the CF transmembrane conductance regulator (CFTR). ΔF508, the most common mutation, is a misfolded protein that is retained in the endoplasmic reticulum and
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9ec0aadb18a49f00c9e3c2f0883e1112
http://europepmc.org/articles/PMC6482459
http://europepmc.org/articles/PMC6482459
Autor:
Inna Sabirzhanova, William B. Guggino, Qiangni Liu, Liudmila Cebotaru, Emily Bergbower, Clément Boinot, Murali K. Yanda
Publikováno v:
Journal of Cystic Fibrosis. 17:582-594
The missing phenylalanine at position 508, located in nucleotide-binding domain (NBD1) of the cystic fibrosis transmembrane regulator (CFTR), is the most common cystic fibrosis mutation. Severe disease-causing mutations also occur in NBD2. To provide
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::59efb62285220a94c48be73432b40d55
https://doi.org/10.1016/j.jcf.2018.05.016
https://doi.org/10.1016/j.jcf.2018.05.016
Publikováno v:
Cellular Physiology and Biochemistry, Vol 41, Iss 6, Pp 2194-2210 (2017)
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology
Background/Aims: Premature degradation of mutated cystic fibrosis transmembrane conductance regulator (CFTR) protein causes cystic fibrosis (CF), the commonest Mendelian disease in Caucasians. Despite recent advances in precision medicines for CF pat
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9805f3caab76ae25cfc1fea5df0af1c5
https://doi.org/10.1159/000475578
https://doi.org/10.1159/000475578
Publikováno v:
Cellular Physiology and Biochemistry, Vol 45, Iss 2, Pp 639-655 (2018)
Background/Aims: The CFTR-Associated Ligand (CAL), a PDZ domain containing protein with two coiled-coil domains, reduces cell surface WT CFTR through degradation in the lysosome by a well-characterized mechanism. However, CAL’s regulatory effect on
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::49101b2bb9b0dcb5f447681557c5e6d5
https://europepmc.org/articles/PMC5861351/
https://europepmc.org/articles/PMC5861351/
Autor:
William B. Guggino, Clément Boinot, Marcelo M. Morales, Liudmila Cebotaru, Inna Sabirzhanova, Miquéias Lopes-Pacheco
Publikováno v:
Journal of Biological Chemistry. 290:25636-25645
Correcting the processing of ΔF508-CFTR, the most common mutation in cystic fibrosis, is the major goal in the development of new therapies for this disease. Here, we determined whether ΔF508 could be rescued by a combination of small-molecule corr
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c2125c6ca4e46a67c3ce9c7a3ca7fa7b
https://doi.org/10.1074/jbc.m115.671925
https://doi.org/10.1074/jbc.m115.671925
Autor:
Clément Boinot, Ann Harris, Isabelle Callebaut, Toby S. Scott-Ward, Michael J. Mutolo, David N. Sheppard, Timea Palmai-Pallag, Zhiwei Cai, Pissared Khuituan, Beihui Liu
Publikováno v:
The Journal of Physiology. 593:2427-2446
Cross-species comparative studies are a powerful approach to understanding the epithelial Cl− channel cystic fibrosis transmembrane conductance regulator (CFTR), which is defective in the genetic disease cystic fibrosis (CF). Here, we investigate t
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::d5686b29370f1c0e7a275fe48a22362b
https://doi.org/10.1113/jp270227
https://doi.org/10.1113/jp270227
Autor:
Clément Boinot, H. Wang, Cristina V. Cebotaru, Inna Sabirzhanova, William B. Guggino, Liudmila Cebotaru
Publikováno v:
Journal of Cystic Fibrosis. 17:S27-S28
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::56147dacbc72be31e38a2068c65290b0
https://doi.org/10.1016/s1569-1993(18)30203-0
https://doi.org/10.1016/s1569-1993(18)30203-0