Results 191 to 200 of about 71,270 (242)
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Cellular and Molecular Life Sciences, 2016
CFTR protein is an ion channel regulated by cAMP-dependent phosphorylation and expressed in many types of epithelial cells. CFTR-mediated chloride and bicarbonate secretion play an important role in the respiratory and gastrointestinal systems. Pharmacological modulators of CFTR represent promising drugs for a variety of diseases.
Zegarra-Moran, Olga +1 more
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CFTR protein is an ion channel regulated by cAMP-dependent phosphorylation and expressed in many types of epithelial cells. CFTR-mediated chloride and bicarbonate secretion play an important role in the respiratory and gastrointestinal systems. Pharmacological modulators of CFTR represent promising drugs for a variety of diseases.
Zegarra-Moran, Olga +1 more
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American Journal of Physiology-Cell Physiology, 1992
Cystic fibrosis (CF) is a fatal genetic disease primarily affecting Caucasians, although cases have been reported from other ethnic groups. CF has a complex etiology, but it is chiefly a disease of electrolyte transport and is characterized by defects in fluid secretion by several epithelia, including the sweat duct, exocrine pancreas, and the ...
C M, Fuller, D J, Benos
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Cystic fibrosis (CF) is a fatal genetic disease primarily affecting Caucasians, although cases have been reported from other ethnic groups. CF has a complex etiology, but it is chiefly a disease of electrolyte transport and is characterized by defects in fluid secretion by several epithelia, including the sweat duct, exocrine pancreas, and the ...
C M, Fuller, D J, Benos
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CFTR haplotype backgrounds on normal and mutant CFTR genes
Human Molecular Genetics, 1994Ten polymorphic loci, located in a 1 Mb interval across the cystic fibrosis locus, were analyzed on normal and mutant CFTR genes. A different distribution of haplotype backgrounds among normal and mutant CFTR genes was observed. With exception of the D7S8 locus, the three most common mutations, delta F508, G542X and N1303K, were found on an identical ...
H, Cuppens +4 more
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The International Journal of Biochemistry & Cell Biology, 2014
Cystic Fibrosis Transmembrane conductance Regulator, CFTR, is a membrane protein expressed in epithelia. A protein kinase A (PKA)-regulated Cl(-) channel, it is a rate-limiting factor in fluid transport. Mutations in CFTR are responsible for cystic fibrosis, CF, an autosomal recessive disease.
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Cystic Fibrosis Transmembrane conductance Regulator, CFTR, is a membrane protein expressed in epithelia. A protein kinase A (PKA)-regulated Cl(-) channel, it is a rate-limiting factor in fluid transport. Mutations in CFTR are responsible for cystic fibrosis, CF, an autosomal recessive disease.
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New England Journal of Medicine, 2003
To the Editor: Wilschanski et al. (Oct. 9 issue)1 report that full-length cystic fibrosis transmembrane conductance regulator (CFTR) protein was expressed more effectively on the surface of nasal epithelial cells after gentamicin treatment than after placebo in patients with cystic fibrosis who had premature stop codons. Bedwell et al.
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To the Editor: Wilschanski et al. (Oct. 9 issue)1 report that full-length cystic fibrosis transmembrane conductance regulator (CFTR) protein was expressed more effectively on the surface of nasal epithelial cells after gentamicin treatment than after placebo in patients with cystic fibrosis who had premature stop codons. Bedwell et al.
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Glutathione permeability of CFTR
American Journal of Physiology-Cell Physiology, 1998The cystic fibrosis transmembrane conductance regulator (CFTR) forms an ion channel that is permeable both to Cl− and to larger organic anions. Here we show, using macroscopic current recording from excised membrane patches, that the anionic antioxidant tripeptide glutathione is permeant in the CFTR channel.
P, Linsdell, J W, Hanrahan
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Pfl�gers Archiv European Journal of Physiology, 2001
Cystic fibrosis (CF) is a complex disease affecting epithelial ion transport. There are not many diseases like CF that have triggered such intense research activities. The complexity of the disease is due to mutations in the CFTR protein, now known to be a Cl(-) channel and a regulator of other transport proteins. The various interactions and the large
Greger, Rainer F +8 more
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Cystic fibrosis (CF) is a complex disease affecting epithelial ion transport. There are not many diseases like CF that have triggered such intense research activities. The complexity of the disease is due to mutations in the CFTR protein, now known to be a Cl(-) channel and a regulator of other transport proteins. The various interactions and the large
Greger, Rainer F +8 more
openaire +3 more sources