Cargando…
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand
Cystic fibrosis (CF), one of the most common lethal genetic diseases, is caused by loss-of-function mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a chloride channel that, when phosphorylated, is gated by ATP. The third most common pathogenic mutation...
Autores principales: | Lin, Wen-Ying, Jih, Kang-Yang, Hwang, Tzyh-Chang |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Rockefeller University Press
2014
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4178940/ https://www.ncbi.nlm.nih.gov/pubmed/25225552 http://dx.doi.org/10.1085/jgp.201411247 |
Ejemplares similares
-
Nonintegral stoichiometry in CFTR gating revealed by a pore-lining mutation
por: Jih, Kang-Yang, et al.
Publicado: (2012) -
CFTR Gating I: Characterization of the ATP-dependent Gating of a Phosphorylation-independent CFTR Channel (ΔR-CFTR)
por: Bompadre, Silvia G., et al.
Publicado: (2005) -
Identification of a novel post-hydrolytic state in CFTR gating
por: Jih, Kang-Yang, et al.
Publicado: (2012) -
Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel
por: Tsai, Ming-Feng, et al.
Publicado: (2010) -
Pharmacological Responses of the G542X-CFTR to CFTR Modulators
por: Fang, Xinxiu, et al.
Publicado: (2022)