Published in Cell Physiol Biochem on November 04, 2009
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Methods for RNA extraction, cDNA preparation and analysis of CFTR transcripts. J Cyst Fibros (2004) 0.89
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. J Cyst Fibros (2004) 0.89
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Immunohistochemistry of CFTR in native tissues and primary epithelial cell cultures. J Cyst Fibros (2004) 0.84
Extended lung cancer incidence follow-up in the Mayo Lung Project and overdiagnosis. J Natl Cancer Inst (2006) 4.39
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet (2013) 2.91
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A KCNH2 branch point mutation causing aberrant splicing contributes to an explanation of genotype-negative long QT syndrome. Heart Rhythm (2008) 1.59
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
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Molecular analysis of Burkholderia cepacia complex isolates from a Portuguese cystic fibrosis center: a 7-year study. J Clin Microbiol (2003) 1.37
Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy. Hum Mol Genet (2002) 1.32
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
Intronic enhancers coordinate epithelial-specific looping of the active CFTR locus. Proc Natl Acad Sci U S A (2009) 1.29
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
HNF1alpha is involved in tissue-specific regulation of CFTR gene expression. Biochem J (2004) 1.25
A complex intronic enhancer regulates expression of the CFTR gene by direct interaction with the promoter. J Cell Mol Med (2009) 1.23
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
An insulator element 3' to the CFTR gene binds CTCF and reveals an active chromatin hub in primary cells. Nucleic Acids Res (2009) 1.19
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
CTCF mediates insulator function at the CFTR locus. Biochem J (2007) 1.16
MicroRNA regulation of expression of the cystic fibrosis transmembrane conductance regulator gene. Biochem J (2011) 1.15
Evaluation of potential regulatory elements identified as DNase I hypersensitive sites in the CFTR gene. Eur J Biochem (2002) 1.14
The CFTR gene and regulation of its expression. Pediatr Pulmonol (2005) 1.07
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
Rescuing mutant CFTR: a multi-task approach to a better outcome in treating cystic fibrosis. Curr Pharm Des (2013) 1.03
Alternative 5' exons of the CFTR gene show developmental regulation. Hum Mol Genet (2003) 1.00
Transcriptional networks driving enhancer function in the CFTR gene. Biochem J (2012) 1.00
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
MUC6 mucin expression inhibits tumor cell invasion. Exp Cell Res (2011) 0.99
The role of the SEA (sea urchin sperm protein, enterokinase and agrin) module in cleavage of membrane-tethered mucins. FEBS J (2005) 0.98
Molecular mechanisms controlling CFTR gene expression in the airway. J Cell Mol Med (2012) 0.97
AMPK controls epithelial Na(+) channels through Nedd4-2 and causes an epithelial phenotype when mutated. Pflugers Arch (2009) 0.97
Exceptionally high representation of Burkholderia cepacia among B. cepacia complex isolates recovered from the major Portuguese cystic fibrosis center. J Clin Microbiol (2007) 0.96
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
RNA structure is a key regulatory element in pathological ATM and CFTR pseudoexon inclusion events. Nucleic Acids Res (2007) 0.96
Regulation of the epithelial Na+ channel by the protein kinase CK2. J Biol Chem (2008) 0.95
N-Glycosylation of the MUC1 mucin in epithelial cells and secretions. Glycobiology (2006) 0.95
A T3 allele in the CFTR gene exacerbates exon 9 skipping in vas deferens and epididymal cell lines and is associated with Congenital Bilateral Absence of Vas Deferens (CBAVD). Hum Mutat (2005) 0.95
Novel regulatory mechanisms for the CFTR gene. Biochem Soc Trans (2009) 0.94
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
The characterization of the first anti-mouse Muc6 antibody shows an increased expression of the mucin in pancreatic tissue of Cftr-knockout mice. Histochem Cell Biol (2010) 0.93
Nucleosome mapping across the CFTR locus identifies novel regulatory factors. Nucleic Acids Res (2013) 0.92
A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein. Mol Cell Probes (2004) 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
Mucin variable number tandem repeat polymorphisms and severity of cystic fibrosis lung disease: significant association with MUC5AC. 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
The mucin MUC4 and its membrane partner ErbB2 regulate biological properties of human CAPAN-2 pancreatic cancer cells via different signalling pathways. PLoS One (2012) 0.91
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
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
The contribution of tandem repeat number to the O-glycosylation of mucins. Glycobiology (2002) 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
Are there any differences in the community acquired pneumonias admitted to hospital over the past decade? Rev Port Pneumol (2010) 0.89
A metabolic and genomic study of engineered Saccharomyces cerevisiae strains for high glycerol production. Metab Eng (2007) 0.89
Atypical 5' splice sites cause CFTR exon 9 to be vulnerable to skipping. Am J Hum Genet (2002) 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
Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth. Am J Physiol Renal Physiol (2012) 0.88
A genome-wide analysis of open chromatin in human tracheal epithelial cells reveals novel candidate regulatory elements for lung function. Thorax (2011) 0.88
Transcriptional regulation of CFTR gene expression. Front Biosci (Elite Ed) (2012) 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
Evaluation of gene targeting by homologous recombination in ovine somatic cells. Mol Reprod Dev (2003) 0.87
[The clinical course of Burkholderia cepacia complex bacteria respiratory infection in cystic fibrosis patients]. Rev Port Pneumol (2008) 0.86
Quantitative methods for the analysis of CFTR transcripts/splicing variants. J Cyst Fibros (2004) 0.86
High-content siRNA screen reveals global ENaC regulators and potential cystic fibrosis therapy targets. Cell (2013) 0.86
DNA polymorphisms in potential regulatory elements of the CFTR gene alter transcription factor binding. Hum Genet (2002) 0.86
Immune mediators regulate CFTR expression through a bifunctional airway-selective enhancer. Mol Cell Biol (2013) 0.85
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies. Respir Res (2013) 0.85
A genome-wide analysis of open chromatin in human epididymis epithelial cells reveals candidate regulatory elements for genes coordinating epididymal function. Biol Reprod (2013) 0.85
Nucleosome occupancy reveals regulatory elements of the CFTR promoter. Nucleic Acids Res (2011) 0.85
Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility. BMC Gastroenterol (2013) 0.85
Collagen XV inhibits epithelial to mesenchymal transition in pancreatic adenocarcinoma cells. PLoS One (2013) 0.85
Hispanic Infants with cystic fibrosis show low CFTR mutation detection rates in the Illinois newborn screening program. J Genet Couns (2012) 0.84
Interaction of intestinal and pancreatic transcription factors in the regulation of CFTR gene expression. Biochim Biophys Acta (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
Validation of an improved pediatric weight estimation strategy. J Pediatr Pharmacol Ther (2013) 0.84
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies. Cell Physiol Biochem (2005) 0.84