منابع مشابه
CFTR and lung homeostasis.
CFTR is a cAMP-activated chloride and bicarbonate channel that is critical for lung homeostasis. Decreases in CFTR expression have dire consequences in cystic fibrosis (CF) and have been suggested to be a component of the lung pathology in chronic obstructive pulmonary disease. Decreases or loss of channel function often lead to mucus stasis, chronic bacterial infections, and the accompanying c...
متن کاملCftr Gene Transfer to Primate Lung
We have evaluated the biological efficacy of El-deleted adenoviruses in baboons for lung-directed gene therapy of cystic fibrosis (CF). T h e experimental design attempted to simulate a phase I clinical trial with animals receiving a single dose of virus to an isolated pulmonary segment. A total of 14 animals divided into four groups, each of which received escalating doses of virus, were used....
متن کاملLung redox homeostasis: emerging concepts.
This symposium was organized to present some aspects of current research pertaining to lung redox function. Focuses of the symposium were on roles of pulmonary endothelial NADPH oxidase, xanthine oxidase (XO)/xanthine dehydrogenase (XDH), heme oxygenase (HO), transplasma membrane electron transport (TPMET), and the zinc binding protein metallothionein (MT) in the propagation and/or protection o...
متن کاملIron homeostasis in the lung.
Iron is essential for many aspects of cellular function. However, it also can generate oxygen-based free radicals that result in injury to biological molecules. For this reason, iron acquisition and distribution are tightly regulated. Constant exposure to the atmosphere results in significant exposure of the lungs to catalytically active iron. The lungs have a mechanism for detoxification to pr...
متن کاملGlucose homeostasis and genotype-phenotype interplay in cystic fibrosis patients with CFTR gene deltaF508 mutation.
OBJECTIVE We sought to determine the clinical phenotype of adolescent/adult patients with cystic fibrosis, according to heterozygosity or homozygosity for cystic fibrosis transmembrane regulator (CFTR) deltaF508 mutation, and to analyze their characteristics according to glucose tolerance status. RESEARCH DESIGN AND METHODS A total of 76 cystic fibrosis patients with CFTR deltaF508 mutation (...
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ژورنال
عنوان ژورنال: American Journal of Physiology-Lung Cellular and Molecular Physiology
سال: 2014
ISSN: 1040-0605,1522-1504
DOI: 10.1152/ajplung.00326.2014