L1117 PDF

Find out more. Like many other works by the artist, this painting was once attributed to Aelbert Cuyp , but it is related to paintings which are now considered to be by Calraet, for example 'Stable Interior with Two Dapple-Grey Horses' Rotterdam, Museum Boymans-van Beuningen. The horse appears, with variations, in the Rotterdam picture. The National Gallery has endeavoured to make as many images of the collection as possible available for non-commercial use. However, an image of this painting is not available to download. This may be due to third party copyright restrictions.

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Cystic fibrosis CF is a fatal genetic disease caused by mutations in cftr, a gene encoding a PKA-regulated Cl - channel. The most common mutation results in a deletion of phenylalanine at position DeltaFCFTR that impairs protein folding, trafficking, and channel gating in epithelial cells.

In the airway, these defects alter salt and fluid transport, leading to chronic infection, inflammation, and loss of lung function. There are no drugs that specifically target mutant CFTR, and optimal treatment of CF may require repair of both the folding and gating defects. Here, we describe two classes of novel, potent small molecules identified from screening compound libraries that restore the function of DeltaFCFTR in both recombinant cells and cultures of human bronchial epithelia isolated from CF patients.

The CFTR-activating effects of the two mechanisms are additive and support the rationale of a drug discovery strategy based on rescue of the basic genetic defect responsible for CF. This site needs JavaScript to work properly. Please enable it to take advantage of the complete set of features! Clipboard, Search History, and several other advanced features are temporarily unavailable. Search: Search. Advanced Clipboard.

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Epub Apr PMID: Cystic fibrosis transmembrane conductance regulator CFTR and renal function. Stanton BA. Wien Klin Wochenschr. PMID: Review. Selective activation of cystic fibrosis transmembrane conductance regulator Cl- and HCO3- conductances. Reddy MM, et al. Liu X, et al. Nat Biotechnol.

Evidence against the rescue of defective DeltaFCFTR cellular processing by curcumin in cell culture and mouse models. Song Y, et al. J Biol Chem. Epub Jul Show more similar articles See all similar articles. Esposito A, et al. Int J Mol Sci. Lopes-Pacheco M. Front Pharmacol.

Discovery of a picomolar potency pharmacological corrector of the mutant CFTR chloride channel. Pedemonte N, et al. Sci Adv. Estabrooks S, Brodsky JL. Estabrooks S, et al. Bose SJ, et al. J Cyst Fibros. Epub Jan 3. Show more "Cited by" articles See all "Cited by" articles. Publication types Research Support, Non-U. Gov't Actions. Animals Actions. Biotinylation Actions. Cell Line Actions. Cells, Cultured Actions. Humans Actions. Ion Channel Gating Actions. Mice Actions. Rats Actions.

Sequence Deletion Actions. Substances Chlorides Actions. Cresols Actions. Pyrazoles Actions. Recombinant Proteins Actions. VRT Actions. Full-text links [x] Atypon. Copy Download.

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