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Cystic fibrosis transmembrane conductance regulator

Cystic Fibrosis Transmembrane Conductance Regulator, CFTR
This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MRP subfamily that is involved in multi-drug resistance. The encoded protein functions as a chloride channel and controls the regulation of other transport pathways. Mutations in this gene are associated with the autosomal recessive disorders cystic fibrosis and congenital bilateral aplasia of the vas deferens. Alternatively spliced transcript variants have been described, many of which result from mutations in this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CAN, HAD, ACID, V1a, AGE
Papers using Cystic Fibrosis Transmembrane Conductance Regulator antibodies
Inefficient replication of Listeria innocua in the cytosol of mammalian cells.
Supplier
Cossart Pascale, In PLoS Pathogens, 2003
... pharmacological inhibitors and radical donors oleamide, carbonoxolone, α-glycerrhetinic acid, brefeldin A, thapsigargin, CFTR inhibitor II (CFTR II.), indomethacin, bumetanide, N-(G)-nitro-L- arginine methyl ester (L-NAME), UO-126, hydrogen peroxide (H2O2) and cumene hydroperoxide were purchased from Sigma Aldrich.
Papers on Cystic Fibrosis Transmembrane Conductance Regulator
Short-chain fatty acids affect cystic fibrosis airway inflammation and bacterial growth.
New
Grasemann et al., Hannover, Germany. In Eur Respir J, 28 Jun 2015
SCFAs also resulted in higher IL-8 release from stimulated cystic fibrosis transmembrane conductance regulator (CFTR) F508del-mutant compared to wild-type CFTR-corrected bronchial epithelial cells.
The differential role of renoguanylin in osmoregulation and apical Cl-/HCO3 - exchange activity in the posterior intestine of the Gulf toadfish (Opsanus beta).
New
Grosell et al., Miami, United States. In Am J Physiol Regul Integr Comp Physiol, 27 Jun 2015
In the posterior intestine, GC-C, as well as the cystic fibrosis transmembrane conductance regulator (CFTR) and basolateral Na(+)/K(+)/2Cl(-)-cotransporter (NKCC1), which comprise a Cl(-)-secretory pathway, were transcriptionally upregulated in 60 ppt.
ANP and CNP activate CFTR expressed in Xenopus laevis oocytes by direct activation of PKA.
New
Forrest et al., New Haven, United States. In J Recept Signal Transduct Res, 27 Jun 2015
OBJECTIVE: We used two-electrode voltage clamping to define the dominant pathway that is employed when natriuretic peptides activate cystic fibrosis transmembrane conductance regulator (CFTR) in the Xenopus oocyte expression system.
Chlorogenic Acid Activates CFTR-Mediated Cl- Secretion in Mice and Humans: Therapeutic Implications for Chronic Rhinosinusitis.
New
Woodworth et al., Birmingham, United States. In Otolaryngol Head Neck Surg, 27 Jun 2015
SUBJECTS AND METHODS: Chlorogenic acid was tested on primary murine nasal septal epithelial (MNSE) (CFTR(+/+) and transgenic CFTR(-/-)) and human sinonasal epithelial (HSNE) (CFTR(+/+) and F508del/F508del) cultures under pharmacologic conditions in Ussing chambers to evaluate effects on transepithelial Cl(-) transport.
Estrogen and progesterone differentially regulate the levels of cystic fibrosis transmembrane regulator (CFTR), adenylate cyclase (AC), and cyclic adenosine mono-phosphate (cAMP) in the rat cervix.
New
Salleh et al., Kuala Lumpur, Malaysia. In Mol Reprod Dev, 26 Jun 2015
UNASSIGNED: The consistency of the cervical mucus changes with the reproductive cycle, which we hypothesized involved changing levels of cystic fibrosis transmembrane regulator (CFTR), adenylate cyclase (AC), and cyclic adenosine mono-phosphate (cAMP).
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
New
Impact
TRAFFIC and TRANSPORT Study Groups. et al., Brisbane, Australia. In N Engl J Med, 17 Jun 2015
UNASSIGNED: Background Cystic fibrosis is a life-limiting disease that is caused by defective or deficient cystic fibrosis transmembrane conductance regulator (CFTR) protein activity.
Another Beginning for Cystic Fibrosis Therapy.
New
Impact
Davis, Cleveland, United States. In N Engl J Med, 17 Jun 2015
Cystic fibrosis, an autosomal recessive disease of epithelial chloride transport, can be caused by more than 1000 mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR).
Translating the genetics of cystic fibrosis to personalized medicine.
Review
New
Guillot et al., Paris, France. In Transl Res, 15 May 2015
This multiorgan disease is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) protein, a chloride channel recognized as regulating several apical ion channels.
The role of pancreatic ducts in the pathogenesis of acute pancreatitis.
Review
New
Rakonczay et al., Szeged, Hungary. In Pancreatology, 07 May 2015
The majority of the review will focus on the central role of cystic fibrosis transmembrane conductance regulator (CFTR), a critical protein in the regulation of ductal secretion, in the pathogenesis of acute pancreatitis which is highlighted by numerous investigations.
Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy.
Review
New
Chmiel et al., Sherbrooke, Canada. In J Cyst Fibros, 23 Apr 2015
Several defective inflammatory responses have been linked to cystic fibrosis transmembrane conductance regulator (CFTR) deficiency including innate and acquired immunity dysregulation, cell membrane lipid abnormalities, various transcription factor signaling defects, as well as altered kinase and toll-like receptor responses.
The draft genome sequence of the ferret (Mustela putorius furo) facilitates study of human respiratory disease.
New
Impact
Katze et al., Seattle, United States. In Nat Biotechnol, Dec 2014
Using microarray data from 16 ferret samples reflecting cystic fibrosis disease progression, we showed that transcriptional changes in the CFTR-knockout ferret lung reflect pathways of early disease that cannot be readily studied in human infants with cystic fibrosis disease.
Physiological impact of abnormal lipoxin A₄ production on cystic fibrosis airway epithelium and therapeutic potential.
Review
New
Urbach et al., Dublin, Ireland. In Biomed Res Int, Dec 2014
In CF, the loss of chloride transport caused by the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel gene results in dehydration, mucus plugging, and reduction of the airway surface liquid layer (ASL) height which favour chronic lung infection and neutrophil based inflammation leading to progressive lung destruction and early death of people with CF.
Long-term safety and efficacy of tobramycin in the management of cystic fibrosis.
Review
New
Ancochea et al., Madrid, Spain. In Ther Clin Risk Manag, Dec 2014
Cystic fibrosis (CF) is a fatal inherited disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene whose mortality is conditioned by a progressive decline in lung function.
Functional repair of CFTR by CRISPR/Cas9 in intestinal stem cell organoids of cystic fibrosis patients.
Impact
Clevers et al., Utrecht, Netherlands. In Cell Stem Cell, 2014
Here we use the CRISPR/Cas9 genome editing system to correct the CFTR locus by homologous recombination in cultured intestinal stem cells of CF patients.
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene.
Impact
Cutting et al., Baltimore, United States. In Nat Genet, 2013
Few of the almost 2,000 variants in the cystic fibrosis transmembrane conductance regulator gene CFTR have empirical evidence that they cause cystic fibrosis.
Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.
GeneRIF
Zabner et al., Iowa City, United States. In Laryngoscope, 2012
CFTR gene has a potential role in the develeopment of chronic sinusitis born with cystic fibrosis .
Conformational changes relevant to channel activity and folding within the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.
GeneRIF
Forman-Kay et al., Toronto, Canada. In J Biol Chem, 2012
Conformational changes relevant to channel activity and folding within the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.
A microRNA network regulates expression and biosynthesis of wild-type and DeltaF508 mutant cystic fibrosis transmembrane conductance regulator.
GeneRIF
McCray et al., Iowa City, United States. In Proc Natl Acad Sci U S A, 2012
miRNA-138 regulates CFTR expression through its interactions with the transcriptional regulatory protein SIN3A.
Combination of CFTR gene mutation and autoimmune pancreatitis presenting as necrotizing pancreatitis.
GeneRIF
Weinstein et al., In Pancreas, 2012
Letter/Case Report: CFTR gene mutations and autoimmune pancreatitis lead to development of necrotizing pancreatitis.
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.
GeneRIF
Hansson et al., Göteborg, Sweden. In J Exp Med, 2012
Data show that the mucus of the small intestine in the CftrDeltaF508 mice is attached to the epithelium and is impenetrable.
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