Article
G551D-CFTR needs more bound actin than wild-type CFTR to maintain its presence in plasma membranes.
Cell biology international - 1 Aug 2015
Trouvé Pascal, Kerbiriou Mathieu, Teng Ling, Benz Nathalie, Taiya Mehdi, Le Hir Sophie, Férec Claude
Abstract excerpt
Cystic Fibrosis is due to mutations in the CFTR gene. The missense mutation G551D (approx. 5% of cases) encodes a CFTR chloride channel with normal cell surface expression but with an altered chloride channel activity, leading to a severe phenotype. Our aim was to identify specific interacting proteins of G551D-CFTR which could explain the channel defect. Wild-type CFTR (Wt-CFTR) was co-immunoprecipitated from...
Topics
- Actins
- Amino Acid Sequence
- Cell Membrane
- Chloride Channels
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Electrophoresis, Gel, Two-Dimensional
- HeLa Cells
- Humans
- Immunoprecipitation
- Mass Spectrometry
- Molecular Sequence Data
- Patch-Clamp Techniques