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CFTR encodes a member of the ATP-binding cassette (ABC) transporter superfamily. Zusätzlich bieten wir Ihnen CFTR Antikörper (234) und CFTR Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.
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This comprehensive CFTR database is now an invaluable tool for diagnostic laboratories gathering information on rare variants, especially in the context of genetic counseling, prenatal and preimplantation genetic diagnosis
this study suggests that CFTR may play a role in the physiology of the innervation of the gastro-intestinal tract
This article reviews the roles of CFTR in epithelial cells, its regulatory role in insulin (zeige INS ELISA Kits) secretion, and a mechanism of CFTR regulation by insulin (zeige INS ELISA Kits). [review]
F508del-homozygosity was far less common among British Asian Cystic Fibrosis (zeige S100A8 ELISA Kits) patients. Only 45% had at least one CFTR mutation included in the UK CF NBS (zeige NBN ELISA Kits) panel (F508del, G551D, 621+1G->T, G542X) against 94% of non-Asians. Thirty percent of Asians had not even one CFTR mutation included in the CFTR panel widely used in UK-for clinical diagnosis, compared to only 1% of non-Asians.
dexa induces a rapid stimulation of CFTR activity which depends on PI3K (zeige PIK3CA ELISA Kits)/AKT (zeige AKT1 ELISA Kits) signaling in airway epithelial cells. Glucocorticoids might thus represent, in addition to their immunomodulatory actions, a therapeutic strategy to rapidly increase airway fluid secretion.
Based on multiple linear regression analysis the dose of vitamin K and F508del mutation were potentially defined as determinants of vitamin K deficiency. In cystic fibrosis (zeige S100A8 ELISA Kits) patients with liver cirrhosis, vitamin K deficiency is not only more frequent, but also more severe. However, not liver cirrhosis, but the presence of a F508del CFTR mutation constitutes an independent risk factor for vitamin K deficiency.
CFTR insertion in the plasma membrane results in local reorganization of actin, PDZ binding proteins and certain GPCRs.
The co-transfection of the pLuc-CFTR-3 UTR (zeige UTS2R ELISA Kits) vector with different combinations of PNAs, miR (zeige MLXIP ELISA Kits)-509-3p, and controls in A549 cells demonstrated the ability of the longer PNA to rescue the luciferase activity by up to 70% of the control, thus supporting the use of suitable PNAs to counteract the reduction in the CFTR expression
beta2-Agonist-induced organoid swelling correlated with the CFTR genotype, and could be induced in homozygous CFTR-F508del organoids and highly differentiated primary cystic fibrosis (zeige S100A8 ELISA Kits) airway epithelial cells after rescue of CFTR trafficking by small molecules
This paper presents an 11-year-old Chinese CF patient with disseminated bronchiectasis and salty sweat. A homozygous deletion involving exon 20 of CFTR was observed in the patient's genome. This study expands the mutation spectrum of CFTR in patients of Chinese origin.
NDPK-A (zeige NME1 ELISA Kits) exists in a functional cellular complex with AMPK (zeige PRKAA2 ELISA Kits) and CFTR in airway epithelia, and NDPK-A (zeige NME1 ELISA Kits) catalytic function is required for the AMPK (zeige PRKAA2 ELISA Kits)-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 (zeige DNAH8 ELISA Kits) 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 ELISA Kits)) transporters.
These data suggest that the Xenopus P2Y1 receptor (zeige P2RY1 ELISA Kits) can increase both cyclic AMP/protein kinase A and calcium/protein kinase C (zeige PKC ELISA Kits) levels and that the PKC (zeige PKC ELISA Kits) 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 ELISA Kits), defect of which leads to premature degradation of beta-catenin (zeige CTNNB1 ELISA Kits) and suppressed activation of beta-catenin (zeige CTNNB1 ELISA Kits) signaling.
CFTR attaches tumor suppressor PTEN to the membrane and promotes anti Pseudomonas aeruginosa immunity.
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 ELISA Kits).
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 ELISA Kits) 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 ELISA Kits) stimulation of Akt1 (zeige AKT1 ELISA Kits) and Akt2 (zeige AKT2 ELISA Kits) signaling in Cystic fibrosis (zeige S100A8 ELISA Kits) airway cells was diminished compared with that observed in airway cells expressing wild-type CFTR.
Biotinylation and streptavidin pull-down assays confirmed that CAL dramatically reduces the expression level of total and cell surface Mrp2 in Huh-7 cells. Our findings suggest that CAL interacts with Mrp2 and is a negative regulator of Mrp2 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 ELISA Kits), via TGF-beta1 (zeige TGFB1 ELISA Kits) receptor I and p38 MAPK (zeige MAPK14 ELISA Kits) 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) 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 ELISA Kits) 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 ELISA Kits) pigs, reveal some spared function in the DeltaF508 vs. null piglets.
causal link between CFTR mutations and partial or total vas (zeige AVP ELISA Kits) 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 ELISA Kits) hypersensitive sites showed high homology in cow and pig.
Results demonstrate functional coupling between Cftr and Slc26a6-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 ELISA Kits)-causing missense mutational sites are identical.
Cftr mutant zebrafish develops pancreatic destruction similar to cystic fibrosis (zeige S100A8 ELISA Kits) 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