[PDF] Top 20 Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives
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Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives
... Cystic fibrosis (CF) is an inherited (recessive autosomal) chronic disease that affects the respiratory, digestive, and reproductive ...the current median age of survival for CF patients is still in ... See full document
14
Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis
... [42], current literature suggests that any clinical benefit is limited by the need for intravenous or intramuscular administration, as well as its lack of potency and potential renal toxicity ... See full document
8
Role of Binding and Nucleoside Diphosphate Kinase A in the Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator by AMP-activated Protein Kinase
... CFTR conductance in oocytes expressing both NDPK-A-H118F and the blocking CFTR-1420-57 peptide ...our current results suggest that AMPK and NDPK lie FIGURE ...cell conductance over time are shown in ... See full document
15
Case Report Chinese data of the CFTR mutation: a report from West China Hospital and literature review
... objective: Cystic fibrosis (CF) is a serious genetic disorder that is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) ...The ... See full document
9
Identification and regulation of the cystic fibrosis transmembrane conductance regulator generated chloride channel
... regulation is due to phosphorylation, because the catalytic subunit of cAMP-dependent protein kinase plus ATP reversibly activated the channel in excised, cell-free patches of membrane. In symmetrical Cl- solutions, the ... See full document
11
Pseudomonas aeruginosa Induced Bleb Niche Formation in Epithelial Cells Is Independent of Actinomyosin Contraction and Enhanced by Loss of Cystic Fibrosis Transmembrane Conductance Regulator Osmoregulatory Function
... The current study showed that CFTR is also in- volved in controlling survival/replication of bacteria after internalization by an epithelial cell, with the mechanism involving its osmoregulatory ... See full document
14
The amiloride inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways
... Cystic fibrosis (CF) airway cells, besides their well-known defect in cAMP-dependent Cl- conductance, are characterized by an enhanced Na+ ...Na+ conductance in human respiratory tract by ... See full document
8
Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR
... molecule modulators of cystic fibrosis transmembrane regulator (CFTR) function for patients with spe- cific genotypes are making a significant impact in the care of patients with ... See full document
11
Challenging the diagnosis of Cystic Fibrosis in a patient carrying the 186-8T/C allelic variant in the CF Transmembrane Conductance Regulator gene
... of mild conditions caused by CFTR dysfunction, associ- ated with limited deterioration over time and clinical man- ifestations in at least one organ in the presence of normal or borderline sweat chloride values. The ... See full document
7
Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis
... for cystic fibrosis (CF) not only identifies infants with a diagnosis of CF, but also those with an uncertain diagnosis of cystic fibrosis (CF), ...CF transmembrane conductance ... See full document
7
Reappraisal of Frequency of Common Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Iranian Cystic Fibrosis Patients
... CFTR gene mutations as well as poly T polymorphisms in 62 CF patients who were registered from the different geographical regions of Iran at the Pediatric Respiratory [r] ... See full document
9
Cystic Fibrosis - An Update
... Cystic fibrosis (CF) is said to be the most common lethal inherited disease of the white ...the cystic fibrosis transmembrane conductance regulator ... See full document
5
The role of cystic fibrosis conductance regulator in the clearance of Burkholderia cenocepacia by macrophages
... Cystic fibrosis transmembrane conductance regulator (CFTR) is a voltage-independent, protein kinase A (PKA)-regulated chloride (Cl - ) channel belonging to the adenosine triphosphate ... See full document
101
Dynamics of soluble and cellular inflammatory markers in nasal lavage obtained from Cystic Fibrosis patients during intravenous antibiotic treatment
... Additionally, no changes in the expression levels of NE, which was detected for the first time in NLF, were observed during the applied therapeutic intervention. Also TNF, MPO, IL-8 and IL-1β remained unchanged during 6 ... See full document
11
Antisense oligodeoxynucleotide to the cystic fibrosis transmembrane conductance regulator inhibits cyclic AMP activated but not calcium activated cell volume reduction in a human pancreatic duct cell line
... Cystic fibrosis (CF) is characterized by a defect in cAMP-regulated chloride channels in epithelial ...CF transmembrane conductance regulator (CFTR) is expressed in the apical membrane ... See full document
6
Chloride conductance and genetic background modulate the cystic fibrosis phenotype of ΔF508 homozygous twins and siblings
... Subjects. The investigated ∆F508 homozygous patient pairs were recruited from a set of 114 CF monozygous and dizygous pairs recruited by European physicians to enroll in the European Cystic Fibrosis Twin ... See full document
12
R248G cystic fibrosis transmembrane conductance regulator mutation in three siblings presenting with recurrent acute pancreatitis and reproductive issues: a case series
... Cystic fibrosis (CF), Online Mendelian Inheritance in Man (OMIM) #219700, is a life-threatening autosomal reces- sive monogenetic disease caused by mutations to the cystic fibrosis ... See full document
5
Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding
... of cystic fibrosis ...the cystic fibrosis transmembrane conductance regulator (CFTR), for example, appear to cause disease by abrogating normal biosynthetic processing, a ... See full document
10
[HCO3-]-regulated expression and activity of soluble adenylyl cyclase in corneal endothelial and Calu-3 cells
... endothelial cells (BCECs), increasing [cAMP] and stimulating PKA, leading to phosphorylation of the cystic fibrosis transmembrane-conductance regulator (CFTR) and increased apical Cl -..[r] ... See full document
12
Localization of the cystic fibrosis transmembrane conductance regulator in pancreas
... transmembrane conductance regulator (CFTR). In this report, antibodies against CFTR peptides were used to localize the CFTR protein in human pancreas. An affinity purified antibody (alpha-1468) ... See full document
6
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