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A stabilizing influence: CAL PDZ inhibition extends the half-life of ΔF508-CFTR.

作者信息

Cushing Patrick R, Vouilleme Lars, Pellegrini Maria, Boisguerin Prisca, Madden Dean R

机构信息

Department of Biochemistry, Dartmouth Medical School, 7200 Vail Building, Hanover, NH 03755-3844, USA.

出版信息

Angew Chem Int Ed Engl. 2010 Dec 17;49(51):9907-11. doi: 10.1002/anie.201005585.

Abstract
摘要

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本文引用的文献

1
Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures.
Am J Physiol Lung Cell Mol Physiol. 2010 Mar;298(3):L304-14. doi: 10.1152/ajplung.00016.2009. Epub 2009 Dec 11.
2
Differential roles of NHERF1, NHERF2, and PDZK1 in regulating CFTR-mediated intestinal anion secretion in mice.
J Clin Invest. 2009 Mar;119(3):540-50. doi: 10.1172/JCI35541. Epub 2009 Feb 16.
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PLoS Biol. 2008 Sep 30;6(9):e239. doi: 10.1371/journal.pbio.0060239.
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The relative binding affinities of PDZ partners for CFTR: a biochemical basis for efficient endocytic recycling.
Biochemistry. 2008 Sep 23;47(38):10084-98. doi: 10.1021/bi8003928. Epub 2008 Aug 29.
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Airway surface dehydration in cystic fibrosis: pathogenesis and therapy.
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Small molecule pharmacological chaperones: From thermodynamic stabilization to pharmaceutical drugs.
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New insights into cystic fibrosis: molecular switches that regulate CFTR.
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Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.
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