Published in J Biol Chem on July 21, 2004
Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest (2005) 4.19
Side chain and backbone contributions of Phe508 to CFTR folding. Nat Struct Mol Biol (2004) 2.43
The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology. Physiol Rev (2012) 1.95
Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating. J Biol Chem (2008) 1.62
Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms. Proc Natl Acad Sci U S A (2006) 1.56
Mechanisms for rescue of correctable folding defects in CFTRDelta F508. Mol Biol Cell (2009) 1.46
The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating. J Physiol (2006) 1.34
Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation. J Gen Physiol (2010) 1.26
Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1. Protein Sci (2010) 1.25
Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2. Mol Biol Cell (2008) 1.23
Inhibiting endoplasmic reticulum (ER)-associated degradation of misfolded Yor1p does not permit ER export despite the presence of a diacidic sorting signal. Mol Biol Cell (2007) 1.21
Restoration of domain folding and interdomain assembly by second-site suppressors of the DeltaF508 mutation in CFTR. FASEB J (2010) 1.18
The V510D suppressor mutation stabilizes DeltaF508-CFTR at the cell surface. Biochemistry (2010) 1.09
Rescue of folding defects in ABC transporters using pharmacological chaperones. J Bioenerg Biomembr (2005) 1.05
Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops. J Biol Chem (2011) 1.04
The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants. Biochem J (2006) 1.02
Mapping of interdomain interfaces required for the functional architecture of Yor1p, a eukaryotic ATP-binding cassette (ABC) transporter. J Biol Chem (2008) 0.98
Label-free DNA sensor based on surface charge modulated ionic conductance. ACS Nano (2009) 0.98
Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Gen Physiol (2011) 0.98
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
Cystic fibrosis transmembrane conductance regulator: a molecular model defines the architecture of the anion conduction path and locates a "bottleneck" in the pore. Biochemistry (2012) 0.94
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant. J Biol Chem (2011) 0.93
Arginines in the first transmembrane segment promote maturation of a P-glycoprotein processing mutant by hydrogen bond interactions with tyrosines in transmembrane segment 11. J Biol Chem (2008) 0.92
Modeling the conformational changes underlying channel opening in CFTR. PLoS One (2013) 0.92
Mechanisms of CFTR Folding at the Endoplasmic Reticulum. Front Pharmacol (2012) 0.90
Interplay between ER exit code and domain conformation in CFTR misprocessing and rescue. Mol Biol Cell (2009) 0.89
A salt bridge in intracellular loop 2 is essential for folding of human p-glycoprotein. Biochemistry (2013) 0.86
The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells. Eur J Hum Genet (2013) 0.85
Molecular determinants dictating cell surface expression of the human sodium-dependent vitamin C transporter-2 in human liver cells. Am J Physiol Gastrointest Liver Physiol (2009) 0.81
Synonymous codon usage affects the expression of wild type and F508del CFTR. J Mol Biol (2015) 0.81
Benzbromarone stabilizes ΔF508 CFTR at the cell surface. Biochemistry (2011) 0.80
Mapping the Binding Site of the Inhibitor Tariquidar That Stabilizes the First Transmembrane Domain of P-glycoprotein. J Biol Chem (2015) 0.79
The cystic-fibrosis-associated ΔF508 mutation confers post-transcriptional destabilization on the C. elegans ABC transporter PGP-3. Dis Model Mech (2012) 0.78
Development of CFTR Structure. Front Pharmacol (2012) 0.77
How Phosphorylation and ATPase Activity Regulate Anion Flux though the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). J Biol Chem (2016) 0.75
Effect of cognitive-existential group therapy on survival in early-stage breast cancer. J Clin Oncol (2004) 2.26
Supportive-expressive group therapy for women with metastatic breast cancer: survival and psychosocial outcome from a randomized controlled trial. Psychooncology (2007) 2.19
Simultaneous binding of two different drugs in the binding pocket of the human multidrug resistance P-glycoprotein. J Biol Chem (2003) 1.79
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein. J Biol Chem (2007) 1.66
Cognitive-existential group psychotherapy for women with primary breast cancer: a randomised controlled trial. Psychooncology (2003) 1.61
Impact of an educational intervention on general practitioners' skills in cognitive behavioural strategies: a randomised controlled trial. Med J Aust (2008) 1.43
Transmembrane segment 7 of human P-glycoprotein forms part of the drug-binding pocket. Biochem J (2006) 1.40
Psychiatric disorder in women with early stage and advanced breast cancer: a comparative analysis. Aust N Z J Psychiatry (2004) 1.39
Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound. Mol Pharm (2005) 1.35
The "LSGGQ" motif in each nucleotide-binding domain of human P-glycoprotein is adjacent to the opposing walker A sequence. J Biol Chem (2002) 1.33
Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants. Biochem J (2007) 1.33
Processing mutations disrupt interactions between the nucleotide binding and transmembrane domains of P-glycoprotein and the cystic fibrosis transmembrane conductance regulator (CFTR). J Biol Chem (2008) 1.32
Location of the rhodamine-binding site in the human multidrug resistance P-glycoprotein. J Biol Chem (2002) 1.28
Substrate-induced conformational changes in the transmembrane segments of human P-glycoprotein. Direct evidence for the substrate-induced fit mechanism for drug binding. J Biol Chem (2003) 1.28
Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones. Mol Pharmacol (2006) 1.26
Transmembrane segment 1 of human P-glycoprotein contributes to the drug-binding pocket. Biochem J (2006) 1.23
Methanethiosulfonate derivatives of rhodamine and verapamil activate human P-glycoprotein at different sites. J Biol Chem (2003) 1.22
Modulating the folding of P-glycoprotein and cystic fibrosis transmembrane conductance regulator truncation mutants with pharmacological chaperones. Mol Pharmacol (2006) 1.21
Drug binding in human P-glycoprotein causes conformational changes in both nucleotide-binding domains. J Biol Chem (2002) 1.18
Predicting P-glycoprotein-mediated drug transport based on support vector machine and three-dimensional crystal structure of P-glycoprotein. PLoS One (2011) 1.17
Identification of residues in the drug translocation pathway of the human multidrug resistance P-glycoprotein by arginine mutagenesis. J Biol Chem (2009) 1.16
Val133 and Cys137 in transmembrane segment 2 are close to Arg935 and Gly939 in transmembrane segment 11 of human P-glycoprotein. J Biol Chem (2004) 1.16
Correctors promote folding of the CFTR in the endoplasmic reticulum. Biochem J (2008) 1.15
Mutational analysis of ABC proteins. Arch Biochem Biophys (2008) 1.14
Disulfide cross-linking analysis shows that transmembrane segments 5 and 8 of human P-glycoprotein are close together on the cytoplasmic side of the membrane. J Biol Chem (2003) 1.13
Human P-glycoprotein is active when the two halves are clamped together in the closed conformation. Biochem Biophys Res Commun (2010) 1.12
Suppressor mutations in the transmembrane segments of P-glycoprotein promote maturation of processing mutants and disrupt a subset of drug-binding sites. J Biol Chem (2007) 1.11
The V510D suppressor mutation stabilizes DeltaF508-CFTR at the cell surface. Biochemistry (2010) 1.09
Psychological morbidity and quality of life in women with advanced breast cancer: a cross-sectional survey. Palliat Support Care (2006) 1.08
Screening for depression in women with metastatic breast cancer: a comparison of the Beck Depression Inventory Short Form and the Hospital Anxiety and Depression Scale. Aust N Z J Psychiatry (2004) 1.07
Rescue of folding defects in ABC transporters using pharmacological chaperones. J Bioenerg Biomembr (2005) 1.05
Guidelines for the psychosocial and bereavement support of family caregivers of palliative care patients. J Palliat Med (2012) 1.04
Do drug substrates enter the common drug-binding pocket of P-glycoprotein through "gates"? Biochem Biophys Res Commun (2005) 1.04
Screening, referral and treatment for depression in patients with coronary heart disease. Med J Aust (2013) 1.03
The Demoralization Scale: a report of its development and preliminary validation. J Palliat Care (2004) 1.02
The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants. Biochem J (2006) 1.02
Introduction of the most common cystic fibrosis mutation (Delta F508) into human P-glycoprotein disrupts packing of the transmembrane segments. J Biol Chem (2002) 1.01
Chemical and pharmacological chaperones as new therapeutic agents. Expert Rev Mol Med (2007) 1.00
Correctors enhance maturation of DeltaF508 CFTR by promoting interactions between the two halves of the molecule. Biochemistry (2009) 1.00
Vanadate trapping of nucleotide at the ATP-binding sites of human multidrug resistance P-glycoprotein exposes different residues to the drug-binding site. Proc Natl Acad Sci U S A (2002) 0.99
Thapsigargin or curcumin does not promote maturation of processing mutants of the ABC transporters, CFTR, and P-glycoprotein. Biochem Biophys Res Commun (2004) 0.99
The ATPase activity of the P-glycoprotein drug pump is highly activated when the N-terminal and central regions of the nucleotide-binding domains are linked closely together. J Biol Chem (2012) 0.97
Corrector VX-809 stabilizes the first transmembrane domain of CFTR. Biochem Pharmacol (2013) 0.97
A review of psychosocial aspects of motor neurone disease. J Neurol Sci (2007) 0.96
Insertion of an arginine residue into the transmembrane segments corrects protein misfolding. J Biol Chem (2006) 0.95
Hazardous or harmful alcohol use in Royal Australian Navy veterans of the 1991 Gulf War: identification of high risk subgroups. Addict Behav (2006) 0.94
Permanent activation of the human P-glycoprotein by covalent modification of a residue in the drug-binding site. J Biol Chem (2003) 0.94
Inhibition of multidrug resistance by adamantylgb3, a globotriaosylceramide analog. J Biol Chem (2007) 0.93
Psychosocial risk factors for coronary heart disease. Med J Aust (2013) 0.93
Disulfiram metabolites permanently inactivate the human multidrug resistance P-glycoprotein. Mol Pharm (2005) 0.92
Arginines in the first transmembrane segment promote maturation of a P-glycoprotein processing mutant by hydrogen bond interactions with tyrosines in transmembrane segment 11. J Biol Chem (2008) 0.92
Rhodamine inhibitors of P-glycoprotein: an amide/thioamide "switch" for ATPase activity. J Med Chem (2009) 0.91
The drug-binding pocket of the human multidrug resistance P-glycoprotein is accessible to the aqueous medium. Biochemistry (2004) 0.91
ATP hydrolysis promotes interactions between the extracellular ends of transmembrane segments 1 and 11 of human multidrug resistance P-glycoprotein. Biochemistry (2005) 0.90
Nucleotide binding, ATP hydrolysis, and mutation of the catalytic carboxylates of human P-glycoprotein cause distinct conformational changes in the transmembrane segments. Biochemistry (2007) 0.88
Supportive-expressive group therapy: the transformation of existential ambivalence into creative living while enhancing adherence to anti-cancer therapies. Psychooncology (2004) 0.88
Human P-glycoprotein contains a greasy ball-and-socket joint at the second transmission interface. J Biol Chem (2013) 0.88
Processing mutations located throughout the human multidrug resistance P-glycoprotein disrupt interactions between the nucleotide binding domains. J Biol Chem (2004) 0.88