مقالات ISI (ترجمه نشده)
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Synthesis and biological evaluation of novel thiazole- VX-809 hybrid derivatives as F508del correctors by QSAR-based filtering tools
Keywords: Aminoarylthiazole; Corrector; QSAR; Cystic fibrosis; CFTR; VX809; CF; cystic fibrosis; CFTR; CF transmembrane conductance regulator; AATs; aminoarylthiazoles; HS-YFP; halide-sensitive yellow fluorescent protein; QSAR; quantitative-structure activity relat
Tools and drugs for uracil nucleotide-activated P2Y receptors
Keywords: ADP; Adenosine 5â²-diphosphate; AP4A; P1,P4-Di(adenosine-5â²)-tetraphosphate; AR; Adenosine receptor; AR-C118925; 5-{[5-(2,8-Dimethyl-5H-dibenzo[a,d]cyclohepten-5-yl)-3,4-dihydro-2-oxo-4-thioxo-1(2H)-pyrimidinyl]methyl}-N-(1H-tetrazol-5-yl)-2-furancarbo
Short CommunicationCorrelation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor
Keywords: CF; cystic fibrosis; CFTR; CF transmembrane conductance regulator; CI; confidence interval; ppFEV1; percent predicted forced expiratory volume in 1Â s; q12h; every 12Â h; Cystic fibrosis; Ivacaftor; Sweat chloride; FEV1;
Short CommunicationMonocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth
Keywords: Inflammation; Monocyte; Macrophage; Lipopolysaccharide; CFTR;
Molecular analysis of exon 13 of cystic fibrosis patients in Middle East: High frequency of K710X mutation
Keywords: CFTR; Cystic fibrosis; K710X; Iran; Middle East;
Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience
Keywords: Cystic fibrosis; Gating mutation; Ivacaftor; p.Ser549Arg (S549R); CF; Cystic Fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; FEV1; forced expiratory volume in one second; FVC; functional vital capacity; FEF25-75%; mid forced expirator
In cellulo analyses of the p.Val322Ala mutation on the CFTR protein conformation and activity
Keywords: In cellulo; CFTR; p.Val322Ala mutation; functionality test; cellular localization;
Research papersBronchocele density in cystic fibrosis as an indicator of allergic broncho-pulmonary aspergillosis: A preliminary study
Keywords: CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; ABPA; allergic broncho-pulmonary aspergillosis; CT-scan; computed tomography scan; HAM; high attenuation mucus; HU; hounsfield units; ROI; region of interest; MDT; multidiscip
Autism presenting in the context of a genetic variant of CFTR and early HSV exposure confounded by chronic pain, altered gut microbiota and paternal abandonment; limitations of current pharmacotherapy and barriers to personalized treatment recommendations
Keywords: Autism; CFTR; HSV; Chronic pancreatitis; Gut microbiota;
Biobanking: towards increased access of biomaterials in cystic fibrosis. Report on the pre-conference meeting to the 13th ECFS Basic Science Conference, Pisa, 30 March-2 April, 2016
Keywords: BALF; broncho-alveolar lavage fluid; BBMRI-ERIC; Biobanking and Biomolecular Resources Research Infrastructure - European Research Infrastructure Consortium; BSWG; Basic Science Working Group; CF; cystic fibrosis; CFF; Cystic Fibrosis Foundation; CFTR;
Original ArticleA multiple reader scoring system for Nasal Potential Difference parameters
Keywords: NPD; Nasal Potential Difference; PD; potential difference; Nasal Potential Difference; CFTR; Clinical trial outcomes;
Original ArticlesEarly-Onset Acute Recurrent and Chronic Pancreatitis Is Associated with PRSS1 or CTRC Gene Mutations
Keywords: pediatric; children; young; risk; genetic; AP; Acute pancreatitis; ARP; Acute recurrent pancreatitis; CFTR; Cystic fibrosis transmembrane conductance regulator; CP; Chronic pancreatitis; CTRC; Chymotrypsin C; EOP; Early-onset pancreatitis; INSPPIRE; INter
Association between F508 deletion in CFTR and chronic pancreatitis risk
Keywords: CFTR; Chronic pancreatitis; F508 deletion; Idiopathic chronic pancreatitis; Meta-analysis;
Original ArticleYear to year change in FEV1 in patients with cystic fibrosis and different mutation classes
Keywords: CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; ECFSPR; European Cystic Fibrosis Society Patient Registry; FEV1; Forced expired volume in 1Â s; Cystic fibrosis; Mutation class; Lung function; Age distribution;
SupplementApplying Cystic Fibrosis Transmembrane Conductance Regulator Genetics and CFTR2 Data to Facilitate Diagnoses
Keywords: CFTR genetic testing; CFTR mutations; CFTR-related disorder; cystic fibrosis; genotype; genotype-phenotype correlation mutation; MVCC; penetrance; variant; CF; Cystic fibrosis; CFMD; CF Mutation Database; CFTR; CF transmembrane conductance regulator; CFTR
CFTR modulates RPS27 gene expression using chloride anion as signaling effector
Keywords: CFTR; RPS27; Chloride; Signaling effector; IL-1β; JNK; Autocrine; IL-1β loop; IL1RN; SP600125;
Corrector VX-809 promotes interactions between cytoplasmic loop one and the first nucleotide-binding domain of CFTR
Keywords: VX-770 (Ivacaftor, Kalydeco) (PubChem CID: 16220172); VX-809 (Lumacaftor) (PubChem CID: 16678941); 15Jf (PubChem CID: 11958611); Cycloheximide (PubChem CID: 6197); CFTR; Cystic fibrosis; VX-809 (Lumacaftor); Bithiazole correctors; Transmembrane- and nucle
Original ArticleCFTR-dependent defect in alternatively-activated macrophages in cystic fibrosis
Keywords: Cystic fibrosis (CF); CFTR; CFTR inhibitors; Monocyte-derived macrophages; Classically activated macrophages; Alternatively activated macrophages); Phagocytosis; Endocytosis; IL-13 receptor;
Original ArticleUse of hyperpolarized helium-3 MRI to assess response to ivacaftor treatment in patients with cystic fibrosis
Keywords: AE; adverse event; BMI; body mass index; CF; cystic fibrosis; CFQ-R; Cystic Fibrosis Questionnaire-Revised; CFTR; CF transmembrane conductance regulator; CT; computed tomography; FEV1; forced expiratory volume in 1Â s; 3He; hyperpolarized helium-3; MRI;
Pregnancy among cystic fibrosis women in the era of CFTR modulators
Keywords: CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; CFFPR; Cystic Fibrosis Foundation Patient Registry; US; United States; FDA; Food and Drug Administration; ANOVA; analysis of variance; GEE; generalized estimating equations; R
Original ArticleAP2 α modulates cystic fibrosis transmembrane conductance regulator function in the human intestine
Keywords: AP2αKD; Knock down of AP2α; CCS; Clathrin-coated structures; C2BBe; Caco2BBe; AP2α; CFTR; Clathrin;
TDP-43 in the spectrum of MND-FTLD pathologies
Keywords: AAV; adeno-associated virus; ALS; amyotrophic lateral sclerosis; BAC; bacterial artificial chromosome; bvFTD; behavioral variant FTD; C9ORF72; chromosome 9 open reading frame 72; CaMKII; Calcium/calmodulin-dependent kinase II; CFTR; cystic fibrosis transm
Original ArticlePharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR
Keywords: AE; adverse event; AUC; area under the curve; BID; twice daily; CF; cystic fibrosis; CFQ-R; Cystic Fibrosis Questionnaire-Revised; CFTR; cystic fibrosis transmembrane conductance regulator; DDI; drug-drug interaction; ECG; electrocardiogram; ER; endopla
Pharmacological chaperone approaches for rescuing GPCR mutants: Current state, challenges, and screening strategies
Keywords: AVP; Arginine Vasopressin; B1R; Bradykinin B1 receptor; CFTR; Cystic fibrosis transmembrane regulator; cNDI; congenital nephrogenic diabetes insipidus; D4R; Dopamine D4 receptor; DERET; diffusion-enhanced resonance energy transfer; ELISA; enzyme- linked i
Original ArticleSensitivity towards the GRP78 inhibitor KP1339/IT-139 is characterized by apoptosis induction via caspase 8 upon disruption of ER homeostasis
Keywords: Sodium trans-[tetrachlorobis(1H-indazole)ruthenate(III)]; Ruthenium; Extrinsic pathway of programmed cell death; GRP78; Caspase 8; 4-PBA; 4-phenylbutyric acid; CHOP; C/EBP-homologous protein; CI; combination index; CFTR; cystic fibrosis transmembrane cond
Functional screening for G protein-coupled receptor targets of 14,15-epoxyeicosatrienoic acid
Keywords: 14,15-EET (EET); 14,15-epoxyeicosatrienoic acids; 14,15-EEZE; 14,15-epoxyeicosa-5(Z)-enoic acid; VSMCs; vascular smooth muscle cells; GPCR; G protein-coupled receptor; CFTR; Cystic Fibrosis Transmembrane Conductance Regulator; PKA; Protein Kinase A; PTG;
Development of therapeutic antibodies to G protein-coupled receptors and ion channels: Opportunities, challenges and their therapeutic potential in respiratory diseases
Keywords: ASM; airway smooth muscle; CFTR; cystic fibrosis transmembrane conductance regulator; COPD; chronic obstructive pulmonary disease; FPR; formyl peptide receptor; GPCR; G protein-coupled receptor; TRP; transient receptor potential; G protein-coupled recepto
Research paperHigh-expressing cystic fibrosis transmembrane conductance regulator interacts with histone deacetylase 2 to promote the development of Ph+ leukemia through the HDAC2-mediated PTEN pathway
Keywords: CFTR; cystic fibrosis transmembrane conductance regulator; HDAC2; histone deacetylase 2; MS; mass spectrometry; ChIP; chromatin immunoprecipitation; LC-MS; liquid chromatography-mass spectrometry; Cystic fibrosis transmembrane conductance regulator (CFT
Tubular lipid binding proteins (TULIPs) growing everywhere
Keywords: BPI; bacterial permeability-inducing protein; CETP; cholesterol ester transfer protein; CFTR; cystic fibrosis transmembrane conductance regulator; DALI; distance matrix alignment; DUF; domain of unknown function; ENaC; epithelial sodium channel; ER; endop
Calumenin contributes to ER-Ca2+ homeostasis in bronchial epithelial cells expressing WT and F508del mutated CFTR and to F508del-CFTR retention
Keywords: CFTR; cystic fibrosis transmembrane conductance regulator; ER; endoplasmic reticulum; CF; cystic fibrosis; SERCA; sarco/endoplasmic reticulum Ca2+ ATPase; PMCA; plasma membrane Ca2+ ATPase; TG; thapsigargin; F508del-CFTR; Calumenin; SERCA; ER Ca2+ homeost
Pathophysiology of Intestinal Na+/H+ Exchange
Keywords: Barrett's Esophagus; Esophageal Adenocarcinoma; Epithelial Injury; Epithelial Restitution; Diarrhea; Infection; Inflammation; Microbiota; Inflammatory Bowel Disease; Hypertension; cAMP; cyclic adenosine monophosphate; CD; Crohn's disease; CFTR; cystic
Research paperCurcumin/poly(2-methyl-2-oxazoline-b-tetrahydrofuran-b-2-methyl-2-oxazoline) formulation: An improved penetration and biological effect of curcumin in F508del-CFTR cell lines
Keywords: Triblock copolymer; Amphiphilic polymer; Curcumin; CFTR; Cystic fibrosis;
Modulation of the TRPV4 ion channel as a therapeutic target for disease
Keywords: 4αPDD; 4α-phorbol 12,13-didecanoate; AHR; airway hyperresponsiveness; AKAP; A-kinase anchor protein; ALI; acute lung injury; BKCa; large conductance calcium-activated potassium channel; CFTR; cystic fibrosis transmembrane conductance regulator; COPD; ch
Protein phosphorylation and its role in the regulation of Annexin A2 function
Keywords: AnxA2; Annexin A2 protein; anxA2; annexin A2 mRNA; CRM1; chromosome region maintenance-1/exportin-1; CFTR; cystic fibrosis transmembrane conductance regulator protein; EGF; epithelial growth factor; HUVEC; human umbilical vein endothelial cells; LmB; Lept
Phenylquinoxalinone CFTR activator as potential prosecretory therapy for constipation
Keywords: CFTR; cystic fibrosis transmembrane conductance regulator; CIC; chronic idiopathic constipation; OIC; opioid-induced constipation; IBS-C; constipation-predominant irritable bowel syndrome; ppm; parts per million; PBS; phosphate-buffered saline; LC/MS; liq
Specific stabilization of CFTR by phosphatidylserine
Keywords: CF; cystic fibrosis; CFTR; cystic fibrosis transmembrane conductance regulator; EGFP; enhanced green fluorescent protein; NBD; nucleotide binding domain; PI; phosphatidylinositol; POPC; 1-palmitoyl-2-oleoyl-phosphatidylcholine; POPE; 1-palmitoyl-2-oleoyl-
Molecular dynamics of the cryo-EM CFTR structure
Keywords: Cystic fibrosis; CFTR; Transmembrane protein structure; Computational biology; Molecular dynamics; CFTR; cystic fibrosis transmembrane conductance regulator; NBD; nucleotide binding domain; MD; molecular dynamics; TH; transmembrane helix; TMD; transmembra
SUMOylation represses the transcriptional activity of the Unfolded Protein Response transducer ATF6
Keywords: ER stress; UPR; ATF6; SUMOylation; CFTR; Transcription;
The relevance of contact-independent cell-to-cell transfer of TDP-43 and SOD1 in amyotrophic lateral sclerosis
Keywords: ALS; amyotrophic lateral sclerosis; CFTR; cystic fibrosis transmembrane conductance regulator; CNS; central nervous system; CSF; cerebrospinal fluid; fALS; familial ALS; FTLD; frontotemporal lobar degeneration; LMN; lower motor neurons; sALS; sporadic ALS
Association of clinical severity of cystic fibrosis with variants in the SLC gene family (SLC6A14, SLC26A9, SLC11A1 and SLC9A3)
Keywords: CFTR; rs17235416; rs17563161; rs3788766; rs7512462; Variability;
Targeted pharmacotherapies for defective ABC transporters
Keywords: 4-PBA; 4-phenylbutyrate; ABC; ATP-Binding Cassette; BRIC2; Benign Recurrent Intrahepatic Cholestasis type 2; BSEP; Bile Salt Export Pump; CF; Cystic Fibrosis; CFTR; Cystic Fibrosis Transmembrane conductance Regulator; CsA; Cyclosporin A; DMSO; DiMethylSul
Interactions and cooperativity between P-glycoprotein structural domains determined by thermal unfolding provides insights into its solution structure and function
Keywords: AMPPCP; β, γ-methylene ATP; CD; circular dichroism; CU; cooperative unit; CFTR; cystic fibrosis transmembrane conductance regulator; DDM; n-dodecyl-β-d-maltopyranoside; ÎHc; calorimetric enthalpy; DLS; dynamic light scattering; DMNG; decyl maltose neo
Pharmacological chaperones for the misfolded melanocortin-4 receptor associated with human obesity
Keywords: AgRP; agouti-related peptide; V2R; vasopressin V2 receptor; CFTR; cystic fibrosis transmembrane conductance regulator; ER; endoplasmic reticulum; GnRHR; gonadotropin-releasing hormone receptor; GPCR; G protein-coupled receptor; Hsc70; cytosolic cognate 70
Short CommunicationNon-allergic asthma as a CFTR-related disorder
Keywords: Asthma; Bronchiectasis; CFTR; Intestinal current measurement; Nasal transepithelial potential difference;
Infectious Chronic Rhinosinusitis
Keywords: Bacterial; Exacerbation; Immunodeficiency; Infectious; Microbiome; Pathogenesis; Rhinosinusitis; Sinonasal; CF; Cystic fibrosis; CFTR; Cystic fibrosis transmembrane conductance regulator; CRS; Chronic rhinosinusitis; CRSsNP; Chronic rhinosinusitis without
Moderate intake of docosahexaenoic acid raises plasma and platelet vitamin E levels in cystic fibrosis patients
Keywords: CF; cystic fibrosis; CFTR; cystic fibrosis trans-membrane conductance regulator; TxB2; thromboxane B2; Docosahexaenoic acid; Antioxidant status; Plasma; Platelet lipids; Oxidative stress;
Pharmacological rescue of mutant CFTR protein improves the viscoelastic properties of CF mucus
Keywords: ALI; air-liquid Interface; ASF; airway surface fluid; BSA; bovine serum albumin; ENaC; epithelial sodium channel; HBE; human bronchial epithelial; MPT; multiple particle tracking; < msd >; mean squared displacement; WGA; wheat germ agglutinin; CFTR; Muc
Epigenetic regulation of CFTR in salivary gland
Keywords: CFTR; Salivary glands; Organ culture; 5-Aza-2â²-deoxycytidine; Epigenetic regulation; DNA methylation;
CFTR and/or pancreatitis susceptibility genes mutations as risk factors of pancreatitis in cystic fibrosis patients?
Keywords: Pancreatitis; Cystic fibrosis; CFTR; Modifier genes; Bicarbonate; Next Generation Sequencing;