Published in Mol Cell Probes on August 01, 2004
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol Cell Biol (2005) 1.58
CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis. Biochemistry (2006) 1.54
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR. J Physiol (2014) 1.00
Interaction between CFTR and prestin (SLC26A5). Biochim Biophys Acta (2010) 0.99
Binding of serum response factor to cystic fibrosis transmembrane conductance regulator CArG-like elements, as a new potential CFTR transcriptional regulation pathway. Nucleic Acids Res (2005) 0.96
Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator. J Biol Chem (2012) 0.84
Antagonistic regulation of cystic fibrosis transmembrane conductance regulator cell surface expression by protein kinases WNK4 and spleen tyrosine kinase. Mol Cell Biol (2011) 0.80
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins. J Biol Chem (2010) 0.78
Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR. Pharmacol Res Perspect (2015) 0.78
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
PTC readthrough in human cells occurs in novel cytoplasmic foci and requires UPF proteins. J Cell Sci (2017) 0.75
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet (2013) 2.91
Transcription-dependent spatial arrangements of CFTR and adjacent genes in human cell nuclei. J Cell Biol (2004) 2.43
Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. Chem Biol (2008) 1.97
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Mol Cell Biol (2005) 1.58
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
Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells. Genes Dev (2007) 1.43
The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70. Biochem J (2002) 1.31
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. Am J Respir Cell Mol Biol (2002) 1.26
Proteomic analysis of nasal cells from cystic fibrosis patients and non-cystic fibrosis control individuals: search for novel biomarkers of cystic fibrosis lung disease. Proteomics (2006) 1.21
A genetic screening strategy identifies novel regulators of the proteostasis network. PLoS Genet (2011) 1.19
Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR. Proc Natl Acad Sci U S A (2007) 1.17
Evaluation of the clinical significance of homB, a novel candidate marker of Helicobacter pylori strains associated with peptic ulcer disease. J Infect Dis (2008) 1.14
Antibodies for CFTR studies. J Cyst Fibros (2004) 1.06
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology (2004) 1.04
Microbial conversion of glycerol to 1,3-propanediol: physiological comparison of a natural producer, Clostridium butyricum VPI 3266, and an engineered strain, Clostridium acetobutylicum DG1(pSPD5). Appl Environ Microbiol (2006) 1.03
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR. J Physiol (2014) 1.00
Biochemical methods to assess CFTR expression and membrane localization. J Cyst Fibros (2004) 1.00
Diagnostic and prognostic biomarker discovery strategies for autoimmune disorders. J Proteomics (2009) 0.99
AMPK controls epithelial Na(+) channels through Nedd4-2 and causes an epithelial phenotype when mutated. Pflugers Arch (2009) 0.97
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi. FEBS J (2013) 0.96
Metabolic engineering of Clostridium acetobutylicum for the industrial production of 1,3-propanediol from glycerol. Metab Eng (2005) 0.96
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein. Biochem Biophys Res Commun (2003) 0.94
Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chem Biol (2013) 0.93
Serum proteomics signature of cystic fibrosis patients: a complementary 2-DE and LC-MS/MS approach. J Proteomics (2010) 0.92
The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients. PLoS One (2011) 0.92
Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis. PLoS One (2012) 0.92
Characterization of novel airway submucosal gland cell models for cystic fibrosis studies. Cell Physiol Biochem (2005) 0.91
Experimental assessment of splicing variants using expression minigenes and comparison with in silico predictions. Hum Mutat (2014) 0.90
Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients. Cell Physiol Biochem (2009) 0.90
CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies. J Histochem Cytochem (2004) 0.90
Methods for RNA extraction, cDNA preparation and analysis of CFTR transcripts. J Cyst Fibros (2004) 0.89
HGF stimulation of Rac1 signaling enhances pharmacological correction of the most prevalent cystic fibrosis mutant F508del-CFTR. ACS Chem Biol (2012) 0.89
Low temperature restoring effect on F508del-CFTR misprocessing: A proteomic approach. J Proteomics (2009) 0.89
A metabolic and genomic study of engineered Saccharomyces cerevisiae strains for high glycerol production. Metab Eng (2007) 0.89
Alcohols, esters and heavy sulphur compounds production by pure and mixed cultures of apiculate wine yeasts. Int J Food Microbiol (2005) 0.89
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth. Am J Physiol Renal Physiol (2012) 0.88
F508del-CFTR increases intracellular Ca(2+) signaling that causes enhanced calcium-dependent Cl(-) conductance in cystic fibrosis. Biochim Biophys Acta (2011) 0.88
Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity. Mol Cell Biol (2011) 0.87
CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector. J Cell Physiol (2008) 0.87
Changes in the proteome of Huh7 cells induced by transient expression of hepatitis D virus RNA and antigens. J Proteomics (2008) 0.86
High-content siRNA screen reveals global ENaC regulators and potential cystic fibrosis therapy targets. Cell (2013) 0.86
Effects of occupational exposure to tobacco smoke: is there a link between environmental exposure and disease? J Toxicol Environ Health A (2013) 0.85
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies. Respir Res (2013) 0.85
Rescue of F508del-CFTR by RXR motif inactivation triggers proteome modulation associated with the unfolded protein response. Biochim Biophys Acta (2010) 0.85
Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility. BMC Gastroenterol (2013) 0.85
Proteomic analysis of naphthalene-induced airway epithelial injury and repair in a cystic fibrosis mouse model. J Proteome Res (2009) 0.84
Cytotoxic gold compounds: synthesis, biological characterization and investigation of their inhibition properties of the zinc finger protein PARP-1. Dalton Trans (2012) 0.84
Immunohistochemistry of CFTR in native tissues and primary epithelial cell cultures. J Cyst Fibros (2004) 0.84
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies. Cell Physiol Biochem (2005) 0.84
Tricarbonyl M(I) (M = Re, (99m)Tc) complexes bearing acridine fluorophores: synthesis, characterization, DNA interaction studies and nuclear targeting. Org Biomol Chem (2010) 0.84
Microarray mRNA expression profiling to study cystic fibrosis. Methods Mol Biol (2011) 0.84
Supramolecular organizations in the aerobic respiratory chain of Escherichia coli. Biochimie (2010) 0.84
Quantification of CFTR transcripts. Methods Mol Biol (2011) 0.83
Applicability of different antibodies for the immunohistochemical localization of CFTR in respiratory and intestinal tissues of human and murine origin. J Histochem Cytochem (2003) 0.83
The replication timing of CFTR and adjacent genes. Chromosome Res (2005) 0.83
What have we learned from mouse models for cystic fibrosis? Expert Rev Mol Diagn (2007) 0.83
Singlet oxygen in antimicrobial photodynamic therapy: photosensitizer-dependent production and decay in E. coli. Molecules (2013) 0.83
Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing. J Cyst Fibros (2004) 0.83
An extract from the medicinal plant Phyllanthus acidus and its isolated compounds induce airway chloride secretion: A potential treatment for cystic fibrosis. Mol Pharmacol (2006) 0.82
Proteome analysis of a human liver carcinoma cell line stably expressing hepatitis delta virus ribonucleoproteins. J Proteomics (2008) 0.81
[(Cp-R)M(CO)3] (M=Re or 99mTc) Arylsulfonamide, arylsulfamide, and arylsulfamate conjugates for selective targeting of human carbonic anhydrase IX. Angew Chem Int Ed Engl (2012) 0.81
Functional genomics assays to study CFTR traffic and ENaC function. Methods Mol Biol (2011) 0.81
Regulation of ENaC biogenesis by the stress response protein SERP1. Pflugers Arch (2012) 0.81
Control of TMEM16A by INO-4995 and other inositolphosphates. Br J Pharmacol (2013) 0.80
Neuronal reprograming of protein homeostasis by calcium-dependent regulation of the heat shock response. PLoS Genet (2013) 0.80
CFTR mutations altering CFTR fragmentation. Biochem J (2013) 0.80
Effect of Annexin A5 on CFTR: regulated traffic or scaffolding? Mol Membr Biol (2010) 0.80
Antagonistic regulation of cystic fibrosis transmembrane conductance regulator cell surface expression by protein kinases WNK4 and spleen tyrosine kinase. Mol Cell Biol (2011) 0.80
Anticancer activity of structurally related ruthenium(II) cyclopentadienyl complexes. J Biol Inorg Chem (2014) 0.80
SELDI-TOF biomarker signatures for cystic fibrosis, asthma and chronic obstructive pulmonary disease. Clin Biochem (2009) 0.80
Ceruloplasmin expression by human peripheral blood lymphocytes: a new link between immunity and iron metabolism. Free Radic Biol Med (2007) 0.80
Pt(II) complexes with bidentate and tridentate pyrazolyl-containing chelators: synthesis, structural characterization and biological studies. Dalton Trans (2011) 0.79
Heat-mediated enrichment of α-synuclein from cells and tissue for assessing post-translational modifications. J Neurochem (2013) 0.79
Non-PCR methods for the analysis of CFTR transcripts. J Cyst Fibros (2004) 0.78
A possible approach for gel-based proteomic studies in recalcitrant woody plants. Springerplus (2013) 0.78
Ulcerogenic Helicobacter pylori strains isolated from children: a contribution to get insight into the virulence of the bacteria. PLoS One (2011) 0.78
Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins. J Biol Chem (2010) 0.78
Novel heterobimetallic radiotheranostic: preparation, activity, and biodistribution. ChemMedChem (2014) 0.77
Design and biological evaluation of new platinum(II) complexes bearing ligands with DNA-targeting ability. Inorg Chem (2014) 0.77