∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis
نویسندگان
چکیده
منابع مشابه
Chemical Rescue of F508-CFTR Mimics Genetic Repair in Cystic Fibrosis Bronchial Epithelial Cells*□S
In a previous study of sodium 4-phenylbutyrate (4-PBA)responsive proteins in cystic fibrosis (CF) IB3-1 bronchial epithelial cells, we identified 85 differentially expressed high abundance proteins from whole cellular lysate (Singh, O. V., Vij, N., Mogayzel, P. J., Jr., Jozwik, C., Pollard, H. B., and Zeitlin, P. L. (2006) Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bro...
متن کاملRescue of F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
Van Goor, Fredrick, Kimberly S. Straley, Dong Cao, Jesús González, Sabine Hadida, Anna Hazlewood, John Joubran, Tom Knapp, Lewis R. Makings, Mark Miller, Timothy Neuberger, Eric Olson, Victor Panchenko, James Rader, Ashvani Singh, Jeffrey H. Stack, Roger Tung, Peter D. J. Grootenhuis, and Paul Negulescu. Rescue of F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures...
متن کاملTrimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.
Cystic fibrosis transmembrane conductance regulator (CFTR) carrying the F508del mutation is retained in endoplasmic reticulum and fails to traffic to the cell surface where it functions as a protein kinase A (PKA)-activated chloride channel. Pharmacological correctors that rescue the trafficking of F508del CFTR may overcome this defect; however, the rescued F508del CFTR still displays reduced c...
متن کاملCalpain Inhibition Promotes the Rescue of F508del-CFTR in PBMC from Cystic Fibrosis Patients
A basal calpain activity promotes the limited proteolysis of wild type (WT) cystic fibrosis conductance regulator (CFTR), inducing the internalization of the split channel. This process contributes to the regulation in the level of the active CFTR at the plasma membranes. In peripheral blood mononuclear cells (PBMC) from 16 healthy donors, the inhibition of calpain activity induces a 3-fold inc...
متن کاملMechanisms for Rescue of Correctable Folding Defects in CFTR F508
Premature degradation of CFTR F508 causes cystic fibrosis (CF). CFTR F508 folding defects are conditional and folding correctors are being developed as CF therapeutics. How the cellular environment impacts CFTR F508 folding efficiency and the identity of CFTR F508’s correctable folding defects is unclear. We report that inactivation of the RMA1 or CHIP ubiquitin ligase permits a pool of CFTR F5...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Nature
سال: 2015
ISSN: 0028-0836,1476-4687
DOI: 10.1038/nature15729