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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, V1a, ACID, 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
Anion conductance selectivity mechanism of the CFTR chloride channel.
New
Linsdell, Halifax, Canada. In Biochim Biophys Acta, Feb 2016
In the cystic fibrosis transmembrane conductance regulator (CFTR) anion-selective channel, both anion relative permeability and anion relative conductance are dependent on anion free energy of hydration - anions that are relatively easily dehydrated tend to show both high permeability and low conductance.
HMGB1 IS INCREASED BY CFTR LOSS OF FUNCTION, IS LOWERED BY INSULIN AND INCREASES IN VIVO AT ONSET OF CFRD.
New
Street et al., Parma, Italy. In J Clin Endocrinol Metab, Feb 2016
OBJECTIVES: To verify HMGB1 levels in CF patients according to glucose tolerance state, and analyze relationships with insulin secretion and resistance.
Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis.
New
Marie et al., Paris, France. In Hum Mol Genet, Feb 2016
UNASSIGNED: Patients with cystic fibrosis (CF) display low bone mass and alterations in bone formation.
Exonic Splicing Mutations Are More Prevalent than Currently Estimated and Can Be Predicted by Using In Silico Tools.
New
Martins et al., Rouen, France. In Plos Genet, Jan 2016
We then used this thoroughly characterized dataset, together with experimental data derived from previous studies on BRCA1, BRCA2, CFTR and NF1, to evaluate the predictive power of 3 in silico approaches recently described as promising tools for pinpointing ESR-mutations.
Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets.
Review
New
Brodlie et al., Newcastle upon Tyne, United Kingdom. In Thorax, Jan 2016
ASL volume depletion in CF is secondary to defective anion transport by the abnormal cystic fibrosis transmembrane conductance regulator protein (CFTR).
∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis.
New
Impact
Yates et al., Los Angeles, United States. In Nature, Jan 2016
Deletion of phenylalanine 508 of the cystic fibrosis transmembrane conductance regulator (∆F508 CFTR) is the major cause of cystic fibrosis, one of the most common inherited childhood diseases.
Streptomycin treatment alters the intestinal microbiome, pulmonary T cell profile and airway hyperresponsiveness in a cystic fibrosis mouse model.
New
Haston et al., Montréal, Canada. In Sci Rep, Dec 2015
Cystic fibrosis transmembrane conductance regulator deficient mouse models develop phenotypes of relevance to clinical cystic fibrosis (CF) including airway hyperresponsiveness, small intestinal bacterial overgrowth and an altered intestinal microbiome.
Divergent signaling via SUMO modification: Potential for CFTR modulation.
Review
New
Frizzell et al., Pittsburgh, United States. In Am J Physiol Cell Physiol, Dec 2015
UNASSIGNED: The cystic fibrosis transmembrane conductance regulator (CFTR) is generally responsible for the cAMP/PKA regulated anion conductance at the apical membranes of secretory epithelial cells.
Hallmarks of therapeutic management of the cystic fibrosis functional landscape.
Review
New
Balch et al., Lisbon, Portugal. In J Cyst Fibros, Nov 2015
The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein does not operate in isolation, rather in a dynamic network of interacting components that impact its synthesis, folding, stability, intracellular location and function, referred to herein as the 'CFTR Functional Landscape (CFFL)'.
Timing of CFTR Pore Opening and Structure of Its Transition State.
New
Impact
Csanády et al., Budapest, Hungary. In Cell, Nov 2015
In CFTR, the chloride ion channel mutated in cystic fibrosis (CF) patients, pore opening is coupled to ATP-binding-induced dimerization of two cytosolic nucleotide binding domains (NBDs) and closure to dimer disruption following ATP hydrolysis.
Exploiting species differences to understand the CFTR Cl- channel.
Review
New
Sheppard et al., Bristol, United Kingdom. In Biochem Soc Trans, Nov 2015
The anion channel cystic fibrosis transmembrane conductance regulator (CFTR) is a unique ATP-binding cassette (ABC) transporter.
Directed differentiation of cholangiocytes from human pluripotent stem cells.
New
Impact
Ghanekar et al., Toronto, Canada. In Nat Biotechnol, Aug 2015
Using three-dimensional culture, the protocol yields cystic and/or ductal structures that express mature biliary markers, including apical sodium-dependent bile acid transporter, secretin receptor, cilia and cystic fibrosis transmembrane conductance regulator (CFTR).
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
New
Impact
TRANSPORT Study Group et al., Brisbane, Australia. In N Engl J Med, Aug 2015
BACKGROUND: Cystic fibrosis is a life-limiting disease that is caused by defective or deficient cystic fibrosis transmembrane conductance regulator (CFTR) protein activity.
Mucociliary dysfunction in HIV and smoked substance abuse.
Review
Unwalla et al., Miami, United States. In Front Microbiol, 2014
Cystic fibrosis transmembrane conductance regulator (CFTR) plays a pivotal role in regulating the periciliary ASL.
The draft genome sequence of the ferret (Mustela putorius furo) facilitates study of human respiratory disease.
Impact
Katze et al., Seattle, United States. In Nat Biotechnol, 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.
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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