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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 29 Mar 2014.

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
Cystic Fibrosis: Unenhanced CT Description of the Appendix in Asymptomatic Adults.
New
Gevenois et al., Brussels, Belgium. In Ajr Am J Roentgenol, 30 Apr 2014
Lung transplant status and CFTR gene mutations were recorded.
A Genomic Signature Approach to Rescue ∆F508-CFTR Biosynthesis and Function.
New
McCray Jr et al., United States. In Am J Respir Cell Mol Biol, 26 Apr 2014
We recently showed that expression of miR-138 enhances CFTR biogenesis and partially rescues CFTR-ΔF508 function in CF airway epithelia.
Aquaporin-4 as a molecular partner of cystic fibrosis transmembrane conductance regulator in rat Sertoli cells.
New
Oliveira et al., Covilhã, Portugal. In Biochem Biophys Res Commun, 19 Apr 2014
The cystic fibrosis transmembrane conductance regulator (CFTR) plays an essential role to male fertility and it was recently suggested that it may promote water transport.
Can a polymorphism in the thalassemia gene and a heterozygote CFTR mutation cause acute pancreatitis?
New
Haas et al., Stockholm, Sweden. In World J Clin Cases, 16 Apr 2014
Sequencing the cystic fibrosis transmembrane conductance regulator (CFTR) gene revealed a heterozygote sequence variant.
Novel regulators of spermatogenesis.
Review
New
Chan et al., Hong Kong, Hong Kong. In Semin Cell Dev Biol, 01 Apr 2014
In this review, we have summarized recent findings on the function and signaling mechanisms of several genes that are known to be associated with disease or pathological processes, including CFTR, CD147, YWK-II and CT genes, and discuss their potential roles in regulating different processes of spermatogenesis.
Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance.
Review
New
Linsdell, Halifax, Canada. In World J Biol Chem, 26 Mar 2014
UNLABELLED: Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel causes cystic fibrosis, while inappropriate activity of this channel occurs in secretory diarrhea and polycystic kidney disease.
Functional repair of CFTR by CRISPR/Cas9 in intestinal stem cell organoids of cystic fibrosis patients.
New
Impact
Clevers et al., Utrecht, Netherlands. In Cell Stem Cell, Jan 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.
Role of the bicarbonate-responsive soluble adenylyl cyclase in pH sensing and metabolic regulation.
Review
New
Oude-Elferink et al., Amsterdam, Netherlands. In Front Physiol, Dec 2013
Additionally, sAC also regulates CFTR channel and plays an important role in regulation of barrier function and apoptosis.
Effects of curcumin on ion channels and transporters.
Review
New
Cai et al., Shanghai, China. In Front Physiol, Dec 2013
The cystic fibrosis transmembrane conductance regulator (CFTR) is a well-studied ion channel target of curcumin.
Lipoxin A4 and Platelet Activating Factor Are Involved in E. coli or LPS-Induced Lung Inflammation in CFTR-Deficient Mice.
New
Su et al., Shanghai, China. In Plos One, Dec 2013
CFTR (cystic fibrosis transmembrane conductance regulator) is expressed by both neutrophils and platelets.
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene.
New
Impact
Cutting et al., Baltimore, United States. In Nat Genet, Oct 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.
A functional CFTR assay using primary cystic fibrosis intestinal organoids.
New
Impact
Beekman et al., Utrecht, Netherlands. In Nat Med, Jul 2013
Here we apply this technology to study primary intestinal organoids of people suffering from cystic fibrosis, a disease caused by mutations in CFTR, encoding cystic fibrosis transmembrane conductance regulator.
Common genetic variants in the CLDN2 and PRSS1-PRSS2 loci alter risk for alcohol-related and sporadic pancreatitis.
Impact
Devlin et al., Pittsburgh, United States. In Nat Genet, 2012
Alcohol was long thought to be the primary causative agent, but genetic contributions have been of interest since the discovery that rare PRSS1, CFTR and SPINK1 variants were associated with pancreatitis risk.
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 .
Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein.
Impact
Rossant et al., Toronto, Canada. In Nat Biotechnol, 2012
Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, which regulates chloride and water transport across all epithelia and affects multiple organs, including the lungs.
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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