NHE3 inhibits PKA-dependent functional expression of CFTR by NHERF2 PDZ interactions
Keywords: CFTR; NHE3; NHERF2; PKA; PDZ domains; Ezrin
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Ambroxol-induced modification of ion transport in human airway Calu-3 epithelia
Keywords: Ion transport; Lung epithelium; Ambroxol; CFTR; β2-Adrenergic agonist
Rab4GTPase modulates CFTR function by impairing channel expression at plasma membrane
Keywords: CFTR; Rab4; Regulation; GTP/GDP exchange; Trafficking
F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive
Keywords: CFTR; Arg-based retention signal; ER exit; Membrane protein assembly; Thermal instability
Critical role of CFTR in uterine bicarbonate secretion and the fertilizing capacity of sperm
Keywords: CFTR; Bicarbonate; Sperm; Capacitation; Endometrium
CFTR gene mutations and asthma in the Norwegian Environment and Childhood Asthma study
Keywords: Asthma; Lung function; Bronchial hyperresponsiveness; Genetic analysis; Cystic fibrosis; CFTR
Prospective Analysis of Cystic Fibrosis Transmembrane Regulator Mutations in Adults With Bronchiectasis or Pulmonary Nontuberculous Mycobacterial Infection
Keywords: airway inflammation; genotype; immunodeficiency; Mycobacterium avium; RNA splicing; BMI; body mass index; CBAVD; congenital bilateral absence of the vas deferens; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane regulator; Dlco; diffusion capacity
Rab27a negatively regulates CFTR chloride channel function in colonic epithelia: Involvement of the effector proteins in the regulatory mechanism
Keywords: CFTR; Rab27a; Trafficking; Regulation; SLP-5; Munc13-4
Aberrant trafficking of a proteolipid protein in a mild Pelizaeus-Merzbacher disease
Keywords: Pelizaeus-Merzbacher disease; proteolipid protein; intracellular trafficking; unfolded protein response; BiP; 78-kDa glucose regulated protein/immunoglobulin heavy chain binding protein; CFTR; cystic fibrosis transmembrane conductance regulator; DYT1 dyst
Relative Contribution of Genetic and Nongenetic Modifiers to Intestinal Obstruction in Cystic Fibrosis
Keywords: CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; DIOS; distal intestinal obstruction syndrome; DZ; dizygous; LOD; logarithm of odds of linkage; MI; meconium ileus; MZ; monozygous; PI; pancreatic insufficiency; PS; pancreatic
Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screening
Keywords: CF; Cystic fibrosis; CFTR; Cystic Fibrosis Transmembrane Conductance Regulator; FN; False-negative; HAZ; Height for age Z score; IRT; Immunoreactive trypsinogen; MI; Meconium ileus; NBS; Newborn screening; PI; Pancreatic insufficient; PS; Pancreatic suffi
A 96-well formatted method for exon and exon/intron boundary full sequencing of the CFTR gene
Keywords: CFTR; Cystic fibrosis; Extended sequencing; High-sensitivity mutational analysis
Exploring the mechanisms of macrolides in cystic fibrosis
Keywords: Azithromycin; Ion transport; Pseudomonas; CFTR; MDR
The inherited genetics of pancreatic cancer and prospects for secondary screening
Keywords: genetics; pancreas cancer; secondary screening; molecular analysis; pancreatic juice; EUROPAC; diabetes mellitus; chronic pancreatitis; cystic fibrosis; CFTR; BRCA1; BRCA2; cytochrome P-450; CT; EUS; ERCP; PSTI; PRSS1; SPINK1; PCR; FAMMM; Peutz-Jeghers sy
A novel missense mutation P1290S at exon-20 of the CFTR gene in a Portuguese patient with congenital bilateral absence of the vas deferens
Keywords: CBAVD; CFTR; DGGE; IVS8-T; cystic fibrosis; male infertility;
Cystic Fibrosis Birth Rates in Canada: A Decreasing Trend since the Onset of Genetic Testing
Keywords: CCFF; Canadian Cystic Fibrosis Foundation; CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator;
Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport
Keywords: CFTR; Cystic fibrosis
Assessing the cost of cystic fibrosis diagnosis and treatment
Keywords: CF; Cystic fibrosis; CFF; Cystic Fibrosis Foundation; CFTR; Cystic fibrosis transmembrane conductance regulator gene; DNA; Deoxyribonucleic acid; FEV1; Forced expiratory volume in 1 second; IRT; Immunoreactive trypsinogen; MI; Meconium ileus; NBS; Newborn
Balancing benefits and risks for cystic fibrosis newborn screening: implications for policy decisions
Keywords: CDC; Center for Disease Control and Prevention; CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; IRT; Immunoreactive trypsinogen; NBS; Newborn screening; PKU; Phenylketonuria;
Spectrum of mutations in CFTR in Finland: 18 years follow-up study and identification of two novel mutations
Keywords: Cystic fibrosis; CFTR; Mutation; Finland;
Diagnostic dilemmas resulting from the immunoreactive trypsinogen/DNA cystic fibrosis newborn screening algorithm
Keywords: CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; [Clâ]; Chloride concentration; DBS; Dried blood spot; IRT; Immunoreactive trypsinogen; NBS; Newborn screening; QNS; Quantity not sufficient;
Reduced exhaled NO is related to impaired nasal potential difference in patients with cystic fibrosis
Keywords: BPD; basal potential difference; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; FENO; concentration of nitric oxide in airways; Na+; sodium; NO; nitric oxide; NOS; nitric oxide synthase; Cystic fibrosis; Ion transport; Nit
Mucoviscidosis
Keywords: Mucoviscidosis; CFTR; Pseudomonas aeruginosa;
A CFTR chloride channel activator prevents HrpNea-induced cell death in Arabidopsis thaliana suspension cells
Keywords: Anion channel; Arabidopsis thaliana; Cell death; CFTR; Harpin; AHAS; acetohydroxyacid synthase; AVD; apoptosis volume decrease; CFTR; cystic fibrosis transmembrane conductance regulator; DMSO; dimethyl sulfoxide; DSEVC; discontinuous single electrode volt
Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes
Keywords: ACMG; American College of Medical Genetics; CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; FN; False-negative results; IRT; Immunoreactive trypsinogen; NBS; Newborn screening;
Detection of CFTR mutations using ARMS and low-density microarrays
Keywords: Microarray; Amplification refractory mutation system; CFTR;
Intraspecific variation in gene expression after seawater transfer in gills of the euryhaline killifish Fundulus heteroclitus
Keywords: Na,K-ATPase; NKCC; CFTR; mRNA expression; Gills; Fish; Ion regulation; Evolution;
A pore-forming toxin produced by Aeromonas sobria activates cAMP-dependent Clâ secretory pathways to cause diarrhea
Keywords: Aeromonas sobria hemolysin; CFTR; Clâ secretion; Colonic epithelial cell;
Docosahexaenoic acid trials in cystic fibrosis: A review of the rationale behind the clinical trials
Keywords: CF; cystic fibrosis; iPLA2; intracellular calcium-independent; CFTR; cystic fibrosis transmembrane conductance regulator; sPLA2; secretory PLA2; FA; fatty acid; cPLA2; cytosolic PLA2; DHA; docosahexaenoic acid; COX; cyclooxygenase; EFA; essential fatty ac
The role of airway epithelium and blood neutrophils in the inflammatory response in cystic fibrosis
Keywords: CFTR; Blood neutrophils; Airway epithelium; Inflammation; Bacterial binding;
Ionic currents in multidrug resistant K562 human leukemic cells
Keywords: CFTR; MDR1; Semi-quantitative PCR; K562 cells; Ion channels; Whole cell currents; DPC-forskolin;
Inhibition of sequestosome 1/p62 up-regulation prevents aggregation of ubiquitinated proteins induced by prostaglandin J2 without reducing its neurotoxicity
Keywords: CFTR; cystic fibrosis transmembrane conductance regulator; COX-1; cyclooxygenase 1; DALIS; dendritic cell aggresome-like-induced structures; DMSO; dimethyl sulfoxide; DRiPs; defective ribosomal products; DTT; dithiothreitol; GFP; green fluorescent protein
A Pilot Study of the Effect of Inhaled Buffered Reduced Glutathione on the Clinical Status of Patients With Cystic Fibrosis
Keywords: aerosolized; bronchiectasis; cystic fibrosis; glutathione; lung function; mucolysis; oxidative stress; BMI; body mass index; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; ELF; epithelial lining fluid; FEF25-75; forced e
Synergic action of insulin and genistein on Na+/K+/2Cl â cotransporter in renal epithelium
Keywords: Epithelial ion transport; CFTR; Short-circuit current;
Regulated vesicle trafficking of membrane transporters in hepatic epithelia
Keywords: Ntcp; Na+/bile salt co-transporter; Bsep; bile salt export pump; Mrp2; multi-drug resistance-related protein 2; Mdr1; multi-drug resistance P-glycoprotein 1; Mdr2; multi-drug resistance P-glycoprotein 2; AQP-8; aquaporin-8 water channels; ASBT; Na+/bile s
Glibenclamide Stimulates Fluid Secretion in Rodent Cholangiocytes Through a Cystic Fibrosis Transmembrane Conductance Regulator-Independent Mechanism
Keywords: BCECF; 2â²7â²-bis(2-carboxyethyl)-5,6,carboxyfluorescein-acetoxymethylester; bp; base pair; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; Cm; membrane capacitance; DIDS; 4,4â²-diisothiocyanato-stilbene-2,2â²-disulfoni
Macromolecular complexes of cystic fibrosis transmembrane conductance regulator and its interacting partners
Keywords: Cystic fibrosis transmembrane conductance regulator; Macromolecular complex; PDZ protein; Interacting partner; ABC; ATP-binding cassette; AFM; atomic force microscopy; AKAP; A-kinase anchoring protein; AMPK; AMP-activated kinase; AP-2; adaptor protein com
A Proinflammatory, Antiapoptotic Phenotype Underlies the Susceptibility to Acute Pancreatitis in Cystic Fibrosis Transmembrane Regulator (â/â) Mice
Keywords: cAMP; adenosine 3â²,5â²-cyclic monophosphate; CCK-8; cholecystokinin octapeptide; CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane regulator; GAPDH; glyceraldehyde phosphate dehydrogenase; ICAM; intercellular adhesion molecule; 125I-CCK-8; iodin
Molecular mechanisms of reduced glutathione transport: role of the MRP/CFTR/ABCC and OATP/SLC21A families of membrane proteins
Keywords: ABC; ATP-binding cassette; Bpt1p; bile pigment transporter-1; BSO; l-buthionine (S,R)-sulfoximine; CFTR; cystic fibrosis transmembrane conductance regulator; DTY167; yeast strain lacking functional Ycf1p; EHBR; Sprague-Dawley Eisai hyperbilirubinuric ra
Transcriptional regulatory networks in lipid metabolism control ABCA1 expression
Keywords: A2AR; adenosine A2A receptor; ABC; ATP-binding cassette; ACAT1; acyl-CoA:cholesterol acyltransferase 1; AP; adapter protein; apo; apolipoprotein; ARL7; ADR-ribosylation factor-like 7; ARNT; arylhydrocarbon receptor nuclear translocator; ATRA; all-trans re
Zinc metabolism in airway epithelium and airway inflammation: basic mechanisms and clinical targets. A review
Keywords: AE; airway epithelium; AEC; airway epithelial cells; AHR; airway hyper-responsiveness; AMG; autometallography; BALF; bronchoalveolar lavage fluid; CDF; cation diffusion facilitator; CFTR; cystic fibrosis transmembrane conductance regulator; Cu/Zn SOD; cop