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- W2024696364 endingPage "202" @default.
- W2024696364 startingPage "190" @default.
- W2024696364 abstract "Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutations in the gene encoding the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Before the discovery 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 the lysosomal enzyme α-glucosidase and a deficiency in the cytosolic enzyme glucose 6-phosphate dehydrogenase. 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." @default.
- W2024696364 created "2016-06-24" @default.
- W2024696364 creator A5043639328 @default.
- W2024696364 creator A5070752219 @default.
- W2024696364 date "2013-01-01" @default.
- W2024696364 modified "2023-10-09" @default.
- W2024696364 title "CFTR activity and mitochondrial function" @default.
- W2024696364 cites W12887499 @default.
- W2024696364 cites W140799337 @default.
- W2024696364 cites W1503847195 @default.
- W2024696364 cites W1505928288 @default.
- W2024696364 cites W1533844265 @default.
- W2024696364 cites W1538940252 @default.
- W2024696364 cites W1573745866 @default.
- W2024696364 cites W1581258387 @default.
- W2024696364 cites W1745204207 @default.
- W2024696364 cites W1775099466 @default.
- W2024696364 cites W1882710607 @default.
- W2024696364 cites W1954895018 @default.
- W2024696364 cites W1965741201 @default.
- W2024696364 cites W1966532097 @default.
- W2024696364 cites W1966962396 @default.
- W2024696364 cites W1968164965 @default.
- W2024696364 cites W1968350473 @default.
- W2024696364 cites W1968561949 @default.
- W2024696364 cites W1969088009 @default.
- W2024696364 cites W1969697265 @default.
- W2024696364 cites W1970251524 @default.
- W2024696364 cites W1970859999 @default.
- W2024696364 cites W1970952563 @default.
- W2024696364 cites W1974323440 @default.
- W2024696364 cites W1974794685 @default.
- W2024696364 cites W1975624285 @default.
- W2024696364 cites W1976906930 @default.
- W2024696364 cites W1977922844 @default.
- W2024696364 cites W1978054295 @default.
- W2024696364 cites W1978368163 @default.
- W2024696364 cites W1979412970 @default.
- W2024696364 cites W1980259507 @default.
- W2024696364 cites W1981120301 @default.
- W2024696364 cites W1981933466 @default.
- W2024696364 cites W1982071077 @default.
- W2024696364 cites W1982148505 @default.
- W2024696364 cites W1982352590 @default.
- W2024696364 cites W1983986241 @default.
- W2024696364 cites W1984380214 @default.
- W2024696364 cites W1984594668 @default.
- W2024696364 cites W1985538633 @default.
- W2024696364 cites W1987121472 @default.
- W2024696364 cites W1988711735 @default.
- W2024696364 cites W1989949255 @default.
- W2024696364 cites W1990344396 @default.
- W2024696364 cites W1990637933 @default.
- W2024696364 cites W1990670389 @default.
- W2024696364 cites W1995240556 @default.
- W2024696364 cites W1995698798 @default.
- W2024696364 cites W1997728584 @default.
- W2024696364 cites W1998195903 @default.
- W2024696364 cites W1998338448 @default.
- W2024696364 cites W1998492933 @default.
- W2024696364 cites W2000166627 @default.
- W2024696364 cites W2001173592 @default.
- W2024696364 cites W2001551679 @default.
- W2024696364 cites W2001587323 @default.
- W2024696364 cites W2002033816 @default.
- W2024696364 cites W2002816571 @default.
- W2024696364 cites W2003027637 @default.
- W2024696364 cites W2004667037 @default.
- W2024696364 cites W2004952182 @default.
- W2024696364 cites W2006513748 @default.
- W2024696364 cites W2008351256 @default.
- W2024696364 cites W2009769910 @default.
- W2024696364 cites W2010058706 @default.
- W2024696364 cites W2010233335 @default.
- W2024696364 cites W2010986573 @default.
- W2024696364 cites W2011923176 @default.
- W2024696364 cites W2012448934 @default.
- W2024696364 cites W2012535637 @default.
- W2024696364 cites W2013099998 @default.
- W2024696364 cites W2013660535 @default.
- W2024696364 cites W2015373462 @default.
- W2024696364 cites W2016241657 @default.
- W2024696364 cites W2016532191 @default.
- W2024696364 cites W2019354571 @default.
- W2024696364 cites W2019450760 @default.
- W2024696364 cites W2019612400 @default.
- W2024696364 cites W2019783448 @default.
- W2024696364 cites W2020142217 @default.
- W2024696364 cites W2020787213 @default.
- W2024696364 cites W2020908516 @default.
- W2024696364 cites W2023026614 @default.
- W2024696364 cites W2023865954 @default.
- W2024696364 cites W2024338143 @default.
- W2024696364 cites W2027248916 @default.
- W2024696364 cites W2027676346 @default.
- W2024696364 cites W2027823440 @default.
- W2024696364 cites W2028616918 @default.
- W2024696364 cites W2030841668 @default.