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Modeling Cystic Fibrosis Patient-Specific Responses to CFTR Modulators Using Human Induced Pluripotent Stem Cells. [PDF]
Am J Respir Crit Care MedKanagarajah KR +9 more
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Correction for Younis et al., CFTR dictates monocyte adhesion by facilitating integrin clustering but not activation. [PDF]
Proc Natl Acad Sci U S Aeuropepmc +1 more source
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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
openaire +3 more sources
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.
openaire +2 more sources