Cftr turnover
WebJul 23, 2014 · When similar studies were applied to primary airway epithelia derived from F508del CFTR homozygous patients, chronic VX-770 administration increased the rate of F508del CFTR turnover and reversed the corrective effects of VX-809 in a dose-dependent manner. The authors also reported that chronic VX-770 exposure had negative effects … WebOct 14, 1994 · Mutant (delta F508) and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) were synthesized initially as an approximately 140-kDa core …
Cftr turnover
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WebNational Center for Biotechnology Information WebAug 30, 2024 · The PM turnover (n = 3) and functional stability (n = 3) of ΔF508-CFTR was determined as in d. The rescued ΔF508-CFTR (26 °C, 48 h) was unfolded for 30 min at …
WebSep 27, 2024 · A number of DUBs regulate the CFTR turnover. USP10, a DUB localized at early endosomes, interacts with WT-CFTR and reduces the CFTR poly-ubiquination in CFBE cells. The USP10-mediated deubiquitination enhances the endocytic recycling of WT-CFTR (Bomberger et al., 2009). The role of USP10 in the PM stability of conformationally … WebFeb 1, 2012 · Effect of 5a on turnover of ΔF508 CFTR at the cell surface. Cell surface labeling was performed on cells expressing ΔF508 CFTR in the presence or absence (Plain) of 20 μM 5a (A). Cells were first incubated for 24 h at 30 °C in the absence (Control) or presence of 5a to promote maturation of the protein.
WebThe cystic fibrosis transmembrane conductance regulator (CFTR) is defective in cystic fibrosis (CF). This protein is a channel that sits on the surface of cells and transports chloride and other molecules, such as bicarbonate. The gene that encodes the CFTR protein, which is also called CFTR, is located on chromosome 7. Mutations in this gene … WebMay 23, 2016 · CFTR turnover. CFTR stability was determined following cycloheximide block (0.2 mg/ml) using following formula: half-life equals elapsed time multiplied by logarithm of two, divided by logarithm of ratio of beginning amount and ending amount (t 1/2 = (elapsed time * log2) / log (beginning amount/ending amount)), as described [23, 24]. …
WebThe cystic fibrosis transmembrane conductance regulator (CFTR) is defective in cystic fibrosis (CF). This protein is a channel that sits on the surface of cells and transports chloride and other molecules, such as …
WebCFTR is listed in the World's largest and most authoritative dictionary database of abbreviations and acronyms CFTR - What does CFTR stand for? The Free Dictionary rug stores morningtonWebOct 25, 2013 · CFTR folding is a complex process, which involves cooperativity between protein domains and occurs both during and after translation . Not surprisingly, CFTR quality control is exerted both during and after translation, as indicated by studies on the turnover rates of CFTR mutant proteins and CFTR ubiquitination during translation (14, 55). The ... rug stores in framingham maWebMar 13, 2015 · In this study, we investigated the involvement of the Cdc42 pathway in CFTR turnover and trafficking in a human bronchiolar epithelial cell line (CFBE41o-) … scarlet red mapleWebAug 1, 2004 · CFTR, a member of the ATP-binding cassette (ABC) transporter superfamily (ABCC7), has 1480 aminoacid residues and comprises several domains: two … scarlet red maple tree informationWebThe first part describes in depth the CFTR structure, processing and regulation of the normal and mutant ion channels. The CFTR gene is affected by more than a thousand of … rug stores in pittsburghWebIt has been proposed that CFTR has additional intracellular functions and that there is altered processing of mutant forms. In examining these functions we found a stable … rug stores in phoenixWebJan 10, 2024 · A second cytosolic E3 ubiquitin ligase, RFFL, is also involved in the turnover of CFTR residing at the cell surface. Unlike CHIP, RFFL is palmitoylated and interacts directly with CFTR, binding through disordered regions independent of molecular chaperones . Ablation of RFFL in cell culture has no effect on the turnover of wild-type … rug stores in shreveport la