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CFTR encodes a member of the ATP-binding cassette (ABC) transporter superfamily. Additionally we are shipping CFTR Kits (34) and CFTR Proteins (7) and many more products for this protein.
Showing 10 out of 233 products:
Chicken Monoclonal CFTR Primary Antibody for BP, FACS - ABIN152670
Walker, Watson, Holmes, Edelman, Banting et al.: Production and characterisation of monoclonal and polyclonal antibodies to different regions of the cystic fibrosis transmembrane conductance regulator (CFTR): detection of immunologically related ... in Journal of cell science 1995
Show all 9 Pubmed References
Human Monoclonal CFTR Primary Antibody for IF, IHC (p) - ABIN5575335
Riordan, Rommens, Kerem, Alon, Rozmahel, Grzelczak, Zielenski, Lok, Plavsic, Chou: Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. in Science (New York, N.Y.) 1989
The most frequent mutation among Karachai Cystic Fibrosis (show S100A8 Antibodies) patients is W1282X (90%).
We identified six different mutations in the four Chinese cystic fibrosis (show S100A8 Antibodies) patients. The c.1766+5G>T, c.595C>T, c.2909G>A, and c.4056G>C had been reported already. The two splicing mutations of c.579+1_579+2insACAT and c.1117-1G>C were novel mutations.
Folate inhibits Hcy-induced ER stress via upregulation of CFTR expression in hepatocytes. Folate inhibits Hcy-induced methylation of CFTR promotor and H3K27me3.
The objective of the present study was : first, to determine which algorithms and molecular analyses are currently used for CFNBS in the United States and, second, to utilize our characterization of the nonwhite CF variant spectrum to analyze the impact of different panels on the detection of CFTR variants in nonwhite CF patients as compared with whites.
The Epithelial Sodium Channel Is a Modifier of the Long-Term Nonprogressive Phenotype Associated with F508del CFTR Mutations.
CFTR heterozygotes with chronic rhinosinusitis have significantly smaller frontal and maxillary sinus size compared to those without mutations, irrespective of disease state.
CFTR polymorphisms are genetic markers that are useful for identifying the mutant CFTR alleles in households of patients with cystic fibrosis (show S100A8 Antibodies) when it is not possible to establish the complete CFTR genotype. Moreover, the polymorphisms can be used for indirect CFTR mutation identification in cases of pre-fertilization, pre-implantation and prenatal analysis.
The effect of complex alleles partially depends on the mutation in trans. Although larger studies are necessary, the CFTR activity on nasal epithelial cells is a rapid contributory tool to classify patients with CFTR dysfunction.
Chloride anion behaves as a signaling effector for CFTR in the modulation of RPS27 (show RPS27 Antibodies) expression.
Variations in CFTR gene is associated with cystic fibrosis (show S100A8 Antibodies).
NDPK-A (show NME1 Antibodies) exists in a functional cellular complex with AMPK (show PRKAA2 Antibodies) and CFTR in airway epithelia, and NDPK-A (show NME1 Antibodies) catalytic function is required for the AMPK (show PRKAA2 Antibodies)-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 (show DNAH8 Antibodies) 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 (show ABCB6 Antibodies)) transporters.
These data suggest that the Xenopus P2Y1 receptor (show P2RY1 Antibodies) can increase both cyclic AMP (show TMPRSS5 Antibodies)/protein kinase A and calcium/protein kinase C (show PKC Antibodies) levels and that the PKC (show PKC Antibodies) pathway is involved in CFTR activation via potentiation of the PKA pathway.
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 (show S100A8 Antibodies).
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 (show GCG Antibodies) 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 (show INS Antibodies) stimulation of Akt1 (show AKT1 Antibodies) and Akt2 (show AKT2 Antibodies) signaling in Cystic fibrosis (show S100A8 Antibodies) airway cells was diminished compared with that observed in airway cells expressing wild-type CFTR.
Biotinylation and streptavidin pull-down assays confirmed that CAL (show S100A11 Antibodies) dramatically reduces the expression level of total and cell surface Mrp2 (show ABCC2 Antibodies) in Huh-7 cells. Our findings suggest that CAL (show S100A11 Antibodies) interacts with Mrp2 (show ABCC2 Antibodies) and is a negative regulator of Mrp2 (show ABCC2 Antibodies) expression.
Myelinosomes secreted from testis somatic TM4 (show TPM4 Antibodies) Sertoli cells provide the release of aggregate-prone mutant, but not normal Huntingtin (Htt (show HTT Antibodies)) exon1. Myelinosomes also support the release of other aggregate-prone mutant protein responsible for cystic fibrosis (show S100A8 Antibodies) (CF), F508delCFTR.
ATP8B1 (show ATP8B1 Antibodies) is important for proper CFTR expression and function.
Loss of cystic fibrosis (show S100A8 Antibodies) transmembrane regulator impairs intestinal oxalate secretion
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 (show TGFB1 Antibodies), via TGF-beta1 (show TGFB1 Antibodies) receptor I and p38 MAPK (show MAPK14 Antibodies) 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 (show SNRPG Antibodies)) 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 (show S100A8 Antibodies) 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 (show S100A8 Antibodies) pigs, reveal some spared function in the DeltaF508 vs. null piglets.
causal link between CFTR mutations and partial or total vas (show AVP Antibodies) 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 (show DNASE1 Antibodies) hypersensitive sites showed high homology in cow and pig.
Results demonstrate functional coupling between Cftr and Slc26a6 (show SLC26A6 Antibodies)-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 (show S100A8 Antibodies)-causing missense mutational sites are identical.
Cftr mutant zebrafish develops pancreatic destruction similar to cystic fibrosis (show S100A8 Antibodies) 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