Cftr turnover
WebMar 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-) expressing wild-type CFTR. Cdc42 is a small GTPase of the Rho family that fulfils numerous cell functions, one of which is endocytosis and recycling process via actin cytoskeleton … WebSnippet: Impaired biosynthetic processing of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR), a cAMP-regulated chloride channel, constitutes the most common cause of CF. Recently, we have identified a distinct category of mutation, caused by premature stop codons and frameshift mutations, which manifests in diminished …
Cftr turnover
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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. WebCFTR recycling was measured by monitoring the reappearance of biotinylated Ab–CFTR complex from the endosomal compartment with NeutrAvidin-HRP, as described in …
WebJun 20, 2013 · Introduction. Cystic fibrosis (CF) is a lethal recessive genetic disorder caused by loss-of-function mutations in the CF transmembrane conductance regulator (CFTR) gene [1–3].The biosynthesis, endocytosis, recycling, and quality control of CFTR are tightly regulated by the ubiquitination system [4–11].CFTR molecules that are misfolded early … WebDec 21, 2012 · As expected from the exceptionally high turnover rate of small intestinal epithelium in comparison with other epithelial tissues, 2–3 days in mice and 5 days in humans , biosynthesis of CFTR in the intestinal crypts must be relatively fast, to explain the kinetics of F508del CFTR rescue by low temperature (maximal at 8 h) or in the presence ...
WebThe CFTR gene encodes an ATP-binding cassette (ABC) transporter that functions as a low conductance Cl(-)-selective channel gated by cycles of ATP binding and hydrolysis at its … 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 …
WebThe temperature dependence of the turnover of the complex-glycosylated wt and T70 CFTR was compared by metabolic pulse-chase studies. The turnover of the complex-glycosylated T70 CFTR was more than fivefold faster at 40ЊC (t 1/2 ‫٠‬ 0.5 h) than at 37ЊC (t 1/2 ‫٠‬ 2.7 h) (Fig. 9, A and C) .
WebJul 31, 2024 · The recent cryo-electron microscopy structures of zebrafish and the human cystic fibrosis transmembrane conductance regulator (CFTR) provided unprecedented insights into putative mechanisms underlying gating of its anion channel activity. Interestingly, despite predictions based on channel activity measurements in biological … meatus of bodyWebAug 13, 2010 · indicate that CFTR turnover is initiated by calpain activation, which is induced by an increased Ca 2 + influx and, following internalization of the cleaved … meatus locationWebThe 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 … pegisys scannerWebNov 7, 2014 · Turnover of CFTR in the apical PM of baby hamster kidney cells overexpressing wtCFTR (BHK-wtCFTR) was also quantified by targeted proteomics based on multiple reaction monitoring mass spectrometry; wtCFTR had a half-life of 29.0 ± 2.5 h in the apical PM. This represents the first direct measurement of CFTR turnover using … pegisys scanner subscriptionWebCalpain has been reported to initiate the turnover of CFTR by cleaving the full-length channel [23]. Although the fragmentation site was not identified, based on the size of the proteolytic ... pegity game rulesWebThe 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 … meatus inferiorWebJul 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 … meatus nasopharyngeal