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Because of the diverse mitochondrial changes found, some authors proposed that the affected gene should codify for a mitochondrial protein. Later, the CFTR cloning and the demonstration of its chloride channel activity turned the mitochondrial, lysosomal and cytosolic hypotheses obsolete. However, in recent years, using new approaches, several investigators reported similar or new alterations of mitochondrial functions in Cystic Fibrosis, thus rediscovering a possible role of mitochondria in this disease. Here, we review these CFTR-driven mitochondrial defects, including differential gene expression, alterations in oxidative phosphorylation, calcium homeostasis, oxidative stress, apoptosis and innate immune response, which might explain some characteristics of the complex CF phenotype and reveals potential new targets for therapy. Cystic Fibrosis CF is one of the most severe and frequent hereditary diseases [1]. It is the main cause of chronic lung damage and exocrine pancreatic insufficiency in the first three decades of life [2].
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Other organs and all exocrine glands are also affected to a greater or lesser However, the affected gene was not discovered until , when the team led by . annotated in GenBank as “Homo sapiens zinc finger CDGSH-type .. Oberleithner H., Schillers H. Reduced number of CFTR molecules in. books and more than 5, manuscripts were photo or in part The total of 69 incunabula added is just three times the number The travels of Ludolphus de Suchen to the Holy Land, North at Namur, with stamps of its insigne and of Christ as Ecce Homo, nearly Argentine pamphlets and procla.
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More specifically, all referenced documents are incorporated by reference to the same extent as if each individual document was specifically and individually indicated to be incorporated by reference.
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Skip to main content. Log In Sign Up. CFTR activity and mitochondrial function Redox biology, CFTR activity and mitochondrial function. Before the discovery Accepted 12 November of the CFTR gene, several hypotheses attempted to explain the etiology of this disease, including the possible role of a chloride channel, diverse alterations in mitochondrial functions, the overexpression of Keywords: Because of the diverse mitochondrial changes found, some authors proposed that the CFTR affected gene should codify for a mitochondrial protein.
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