Published in J Physiol on February 16, 2006
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci U S A (2011) 3.87
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Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization. J Biol Chem (2015) 0.97
Functional arrangement of the 12th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant. Pflugers Arch (2011) 0.96
The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction. FASEB J (2013) 0.93
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Genetic selection for a highly functional cysteine-less membrane protein using site saturation mutagenesis. Anal Biochem (2007) 0.85
A stable human-cell system overexpressing cystic fibrosis transmembrane conductance regulator recombinant protein at the cell surface. Mol Biotechnol (2015) 0.83
Cysteine accessibility probes timing and extent of NBD separation along the dimer interface in gating CFTR channels. J Gen Physiol (2015) 0.83
Role of the juxtamembrane region of cytoplasmic loop 3 in the gating and conductance of the cystic fibrosis transmembrane conductance regulator chloride channel. Biochemistry (2012) 0.80
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Using a cysteine-less mutant to provide insight into the structure and mechanism of CFTR. J Physiol (2006) 0.77
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Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. Mol Biol Cell (2005) 2.13
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Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic (2008) 1.34
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Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases. Pflugers Arch (2010) 1.08
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Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator. J Physiol (2009) 1.06
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Computational studies reveal phosphorylation-dependent changes in the unstructured R domain of CFTR. J Mol Biol (2008) 1.01
Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci U S A (2003) 1.00
Biochemical methods to assess CFTR expression and membrane localization. J Cyst Fibros (2004) 1.00
Pioglitazone Attenuates Cystic Burden in the PCK Rodent Model of Polycystic Kidney Disease. PPAR Res (2010) 0.99
Novel regulation of cystic fibrosis transmembrane conductance regulator (CFTR) channel gating by external chloride. J Biol Chem (2004) 0.99
Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysis. FEBS Lett (2002) 0.98
ATP binding to the first nucleotide-binding domain of multidrug resistance protein MRP1 increases binding and hydrolysis of ATP and trapping of ADP at the second domain. J Biol Chem (2001) 0.95
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Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling. Biochim Biophys Acta (2010) 0.93
Role of individual R domain phosphorylation sites in CFTR regulation by protein kinase A. Biochim Biophys Acta (2009) 0.92
ATP binding, not hydrolysis, at the first nucleotide-binding domain of multidrug resistance-associated protein MRP1 enhances ADP.Vi trapping at the second domain. J Biol Chem (2002) 0.92
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implications. Biochem J (2002) 0.91
Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains. Protein Sci (2014) 0.91
The C-terminus of the transmembrane mucin MUC17 binds to the scaffold protein PDZK1 that stably localizes it to the enterocyte apical membrane in the small intestine. Biochem J (2008) 0.90
Role of fatty acid synthase in gemcitabine and radiation resistance of pancreatic cancers. Int J Biochem Mol Biol (2011) 0.90
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Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. J Cyst Fibros (2004) 0.89
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AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis. PLoS Pathog (2011) 0.87
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Nucleotide dissociation from NBD1 promotes solute transport by MRP1. Biochim Biophys Acta (2005) 0.85
A 61-year-old woman with osteomalacia and a thoracic spine lesion. Brain Pathol (2010) 0.83
Imaging CFTR protein localization in cultured cells and tissues. Methods Mol Biol (2011) 0.82
Survival after resection for invasive intraductal papillary mucinous neoplasm and for pancreatic adenocarcinoma: a multi-institutional comparison according to American Joint Committee on Cancer Stage. J Am Coll Surg (2011) 0.82
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Fluorescent modified phosphatidylcholine floppase activity of reconstituted multidrug resistance-associated protein MRP1. Biochim Biophys Acta (2004) 0.80
Targeted proteomic quantitation of the absolute expression and turnover of cystic fibrosis transmembrane conductance regulator in the apical plasma membrane. J Proteome Res (2014) 0.77
Erratum to: A Stable Human-Cell System Overexpressing Cystic Fibrosis Transmembrane Conductance Regulator Recombinant Protein at the Cell Surface. Mol Biotechnol (2015) 0.76
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