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CFTR encodes a member of the ATP-binding cassette (ABC) transporter superfamily. Zusätzlich bieten wir Ihnen CFTR Antikörper (233) und CFTR Kits (35) und viele weitere Produktgruppen zu diesem Protein an.
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study's results help fill in knowledge gaps about particular CFTR variants. More than 2,000 different CF mutations have been reported, but according to a widely used database called Clinical and Functional Translation of CFTR (CFTR2), only 10% are categorized as disease-causing
The p.H609R was found cystic fibrosis (zeige S100A8 Proteine)-causing mutations in homozygous state in four patients with cystic fibrosis (zeige S100A8 Proteine) symptoms.
Sweat chloride and respiratory symptom scores improved with lumacaftor/ivacaftor, though no meaningful benefit was seen in ppFEV1 or body mass index in patients heterozygous for F508del-CFTR.
Authors determined the amino acids inserted at the cystic fibrosis transmembrane conductance regulator (CFTR) W1282X PTC (zeige F9 Proteine) (a UGA codon) in the context of its three upstream and downstream CFTR codons during G418-mediated suppression.
CFTR is strongly expressed in sebaceous glands; in the eccrine glands and ducts, CFTR is located on the apical side of the membrane
In patients with Cystic Fibrosis (zeige S100A8 Proteine) and G551D mutation Extra-esophageal reflux may lead to respiratory microaspiration and probably cause the deterioration of lung function.
In patients with Cystic Fibrosis (zeige S100A8 Proteine) and a G551D CFTR mutation, we demonstrated substantial variability of the sweat chloride concentrations.
Heme-independent activation of the CFTR channel and the Slo1 BKCa (zeige KCNMA1 Proteine) channel by carbon monoxide (CO) may represent two different modes of gating regulation of ion channels by CO. In the case of CFTR, Fe3+ is bound to the R-ICL3 interface.15,16 This interfacial Fe3+ site is formed by traditional coordination ligands such as H950 and H954 from ICL3 and C832 and D836 from the R domain. [Review]
Endogenous oestrogen upregulates the expressions and functional activities of CFTR and SLC26A6 (zeige SLC26A6 Proteine) in duodenal mucosa.
CFTR attaches tumor suppressor PTEN to the membrane and promotes anti Pseudomonas aeruginosa immunity.
NDPK-A (zeige NME1 Proteine) exists in a functional cellular complex with AMPK (zeige PRKAA2 Proteine) and CFTR in airway epithelia, and NDPK-A (zeige NME1 Proteine) catalytic function is required for the AMPK (zeige PRKAA2 Proteine)-dependent regulation of CFTR
Study conclude that when both CFTR and NPT2a are expressed in X. laevis oocytes, CFTR confers to NPT2a a cAMPi-dependent trafficking to the membrane.
wild-type CFTR channel gating cycle is essentially irreversible and tightly coupled to the ATPase cycle, and that this coupling is completely destroyed by the NBD2 Walker B mutation D1370N but only partially disrupted by the NBD1 Walker A mutation K464A.
The cystic fibrosis transmembrane conductance regulator (CFTR) is a protein that belongs to the superfamily of ATP binding cassette (ABC (zeige ABCB6 Proteine)) transporters.
These data suggest that the Xenopus P2Y1 receptor (zeige P2RY1 Proteine) can increase both cyclic AMP (zeige TMPRSS5 Proteine)/protein kinase A and calcium/protein kinase C levels and that the PKC pathway is involved in CFTR activation via potentiation of the PKA pathway.
these results demonstrate that CFTR prevents inflammation and atherogenesis via inhibition of NFkappaB and MAPKs activation
CFTR physically interacts with beta-catenin (zeige CTNNB1 Proteine), defect of which leads to premature degradation of beta-catenin (zeige CTNNB1 Proteine) and suppressed activation of beta-catenin (zeige CTNNB1 Proteine) signaling.
Our studies reveal a novel function for CFTR in antiviral immunity and demonstrate that the DeltaF508 mutation in cftr is coupled to an impaired adaptive immune response. This important insight could open up new approaches for patient care and treatment.
a new and more convenient approach, based on in vivo imaging analysis, has been set up to evaluate the inflammatory response in the lung of CFTR-deficient (CF) mice, a murine model of cystic fibrosis (zeige S100A8 Proteine).
results reveal that by potentiating adenosine triphosphate-sensitive K+ (KATP) channel, cystic fibrosis transmembrane conductance regulator CFTR acts as a glucose-sensing negative regulator of glucagon (zeige GCG Proteine) secretion in alpha cells
This study demonstrates that CFTR plays an important role in tenogenic differentiation and tendon regeneration by inhibiting the beta-catinin/pERK1/2 signaling pathway.
CFTR is a tumor suppressor gene in murine and human colorectal cancer
Insulin (zeige INS Proteine) stimulation of Akt1 (zeige AKT1 Proteine) and Akt2 (zeige AKT2 Proteine) signaling in Cystic fibrosis (zeige S100A8 Proteine) airway cells was diminished compared with that observed in airway cells expressing wild-type CFTR.
Biotinylation and streptavidin pull-down assays confirmed that CAL (zeige S100A11 Proteine) dramatically reduces the expression level of total and cell surface Mrp2 (zeige ABCC2 Proteine) in Huh-7 cells. Our findings suggest that CAL (zeige S100A11 Proteine) interacts with Mrp2 (zeige ABCC2 Proteine) and is a negative regulator of Mrp2 (zeige ABCC2 Proteine) expression.
Results suggest that acetylcholine does not regulate the activity of the CFTR in tracheal epithelia of pigs which opposes observation from studies using mice airway epithelium.
Expression of CFTR-F508del interferes with smooth muscle cell calcium handling and decreases aortic responsiveness.
Pseudomonas aeruginosa and other bacteria into the lumen of intact isolated swine tracheas triggers CFTR-dependent airway surface liquid secretion by the submucosal glands.
TGF-beta1 (zeige TGFB1 Proteine), via TGF-beta1 (zeige TGFB1 Proteine) receptor I and p38 MAPK (zeige MAPK14 Proteine) signaling, reduces CFTR expression to impair CFTR-mediated anion secretion, which would likely compound the effects associated with mild CFTR mutations and ultimately would compromise male fertility.
The esophageal submucosal glands (SMG (zeige SNRPG Proteine)) secrete HCO(3)(-) and mucus into the esophageal lumen, where they contribute to acid clearance and epithelial protection. We investigated the presence of CFTR, its involvement in the secretion process, and the effect of cAMP on HCO3 secretion in this tissue. This is the first report on the presence of CFTR channels in the esophagus.
data suggest that loss of CFTR directly alters Schwann cell function and that some nervous system defects in people with cystic fibrosis (zeige S100A8 Proteine) are likely primary
The data suggest, that during bacterial infections and resulting release of proinflammatory cytokines, the glands are stimulated to secrete fluid, and this response is mediated by cAMP-activated CFTR.
CFTR is required for maximal liquid absorption by lung alveoli under cAMP stimulation
These findings reveal differences between nasal and tracheal glands, show defective fluid secretion in nasal glands of cystic fibrosis (zeige S100A8 Proteine) pigs, reveal some spared function in the DeltaF508 vs. null piglets.
causal link between CFTR mutations and partial or total vas (zeige AVP Proteine) deferens and/or epididymis atresia at birth
conserved CFTR sequences between species are examined for potential regulatory elements. Regions of introns 2, 3, 10, 17a, 18, and 21 and 3' flanking sequence corresponding to human CFTR DNase I (zeige DNASE1 Proteine) hypersensitive sites showed high homology in cow and pig.
Results demonstrate functional coupling between Cftr and Slc26a6 (zeige SLC26A6 Proteine)-like Cl(-)/HCO(3)(-) exchange activity in apical membrane of guinea pig pancreatic interlobular duct.
Molecular dynamics of the cryo-EM CFTR structure
Study presents the structure of zebrafish CFTR in the phosphorylated, ATP-bound conformation, determined by cryoelectron microscopy to 3.4 A resolution. Comparison of the two conformations shows major structural rearrangements leading to channel opening.
Study determined the structure of the zebrafish CFTR by electron cryo-microscopy to 3.7 A resolution; shares 55% sequence identity with human CFTR, and 42 of the 46 cystic-fibrosis (zeige S100A8 Proteine)-causing missense mutational sites are identical.
Cftr mutant zebrafish develops pancreatic destruction similar to cystic fibrosis (zeige S100A8 Proteine) of the human pancreas.
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.
cAMP-dependent chloride channel
, channel conductance-controlling ATPase
, cystic fibrosis transmembrane conductance regulator
, cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7)
, cystic fibrosis transmembrane conductance regulator, ATP-binding cassette (sub-family C, member 7)
, ATP-binding cassette sub-family C member 7
, ATP-binding cassette transporter sub-family C member 7
, ATP-binding cassette, subfamily c, member 7
, cystic fibrosis transmembrane conductance regulator homolog
, cystic fibrosis transmembrane conductance regulator homolog; ATP-binding cassette, subfamily c, member 7
, CFTR chloride channel
, chloride channel
, CFTR cAMP-dependent chloride channel protein
, Channel conductance-controlling ATPase