Matches in SemOpenAlex for { <https://semopenalex.org/work/W51931279> ?p ?o ?g. }
- W51931279 endingPage "258" @default.
- W51931279 startingPage "229" @default.
- W51931279 abstract "Cystic fibrosis (CF) is a complex, fatal autosomal recessive inherited disorder caused by mutations of the CF transmembrane conductance regulator (CFTR) gene [1]. The disease affects children and young adults and is most common in individuals of Northern European descent, with an estimated incidence in Caucasians of 1 in 2,500 births and a prevalence of 30,000 in the United States. Remarkably, the heterozygous carrier frequency in this population is 1 in 25 individuals. CF is also found in individuals of African and Southern European descent, Native Americans and Ashkenazi Jews although it is significantly less frequent in these populations [1, 2]. The first comprehensive clinical descriptions of CF as a familial disorder were provided in the 1930s [3, 4] followed by identification of the autosomal recessive mode of inheritance in 1946 [5]. A biochemical clue came in 1953 when an abnormally high sodium chloride concentration was noted in the sweat of individuals with CF [6]. Additional biochemical insight was provided in the 1980s with the description of abnormal regulation of cyclic adenosine monophosphate (cAMP)-activated chloride conductance in the apical membrane of epithelial cells of CF sweat glands [7] and respiratory airways [8]." @default.
- W51931279 created "2016-06-24" @default.
- W51931279 creator A5027381383 @default.
- W51931279 date "1997-01-01" @default.
- W51931279 modified "2023-09-24" @default.
- W51931279 title "Gene Therapy for Cystic Fibrosis Lung Disease" @default.
- W51931279 cites W1271675486 @default.
- W51931279 cites W1484126091 @default.
- W51931279 cites W1513908459 @default.
- W51931279 cites W1520845559 @default.
- W51931279 cites W1533089062 @default.
- W51931279 cites W1554379134 @default.
- W51931279 cites W1557739264 @default.
- W51931279 cites W1559506200 @default.
- W51931279 cites W1562601302 @default.
- W51931279 cites W1605262540 @default.
- W51931279 cites W1648736195 @default.
- W51931279 cites W1756236371 @default.
- W51931279 cites W1857650329 @default.
- W51931279 cites W1963741984 @default.
- W51931279 cites W1965812300 @default.
- W51931279 cites W1967187884 @default.
- W51931279 cites W1969697265 @default.
- W51931279 cites W1970417334 @default.
- W51931279 cites W1970586355 @default.
- W51931279 cites W1970830865 @default.
- W51931279 cites W1971425598 @default.
- W51931279 cites W1975971473 @default.
- W51931279 cites W1985696897 @default.
- W51931279 cites W1986769432 @default.
- W51931279 cites W1990561446 @default.
- W51931279 cites W1990637933 @default.
- W51931279 cites W1990991203 @default.
- W51931279 cites W199184835 @default.
- W51931279 cites W1992811541 @default.
- W51931279 cites W1993207965 @default.
- W51931279 cites W1993935349 @default.
- W51931279 cites W1995240556 @default.
- W51931279 cites W1997182007 @default.
- W51931279 cites W1998130586 @default.
- W51931279 cites W1999157369 @default.
- W51931279 cites W2008785883 @default.
- W51931279 cites W2010123009 @default.
- W51931279 cites W2011727906 @default.
- W51931279 cites W2013002972 @default.
- W51931279 cites W2013306854 @default.
- W51931279 cites W2014019711 @default.
- W51931279 cites W2015617337 @default.
- W51931279 cites W2016200465 @default.
- W51931279 cites W2016532191 @default.
- W51931279 cites W2019612944 @default.
- W51931279 cites W2020301682 @default.
- W51931279 cites W2022322532 @default.
- W51931279 cites W2023243075 @default.
- W51931279 cites W2025728946 @default.
- W51931279 cites W2026036946 @default.
- W51931279 cites W2028171116 @default.
- W51931279 cites W2030302585 @default.
- W51931279 cites W2033909465 @default.
- W51931279 cites W2034091304 @default.
- W51931279 cites W2036921663 @default.
- W51931279 cites W2038453307 @default.
- W51931279 cites W2039033043 @default.
- W51931279 cites W2039599894 @default.
- W51931279 cites W2043502964 @default.
- W51931279 cites W2044275765 @default.
- W51931279 cites W2046103803 @default.
- W51931279 cites W2047963123 @default.
- W51931279 cites W2048303863 @default.
- W51931279 cites W2050573110 @default.
- W51931279 cites W2054302169 @default.
- W51931279 cites W2054878970 @default.
- W51931279 cites W2056262259 @default.
- W51931279 cites W2058093119 @default.
- W51931279 cites W2060216304 @default.
- W51931279 cites W2062363361 @default.
- W51931279 cites W2067157368 @default.
- W51931279 cites W2071738676 @default.
- W51931279 cites W2071970498 @default.
- W51931279 cites W2072054870 @default.
- W51931279 cites W2078380427 @default.
- W51931279 cites W2078920860 @default.
- W51931279 cites W2079209506 @default.
- W51931279 cites W2079791074 @default.
- W51931279 cites W2083283122 @default.
- W51931279 cites W2084149528 @default.
- W51931279 cites W2084338677 @default.
- W51931279 cites W2084506241 @default.
- W51931279 cites W2085490249 @default.
- W51931279 cites W2085511152 @default.
- W51931279 cites W2089708292 @default.
- W51931279 cites W2090864647 @default.
- W51931279 cites W2091833091 @default.
- W51931279 cites W2093082335 @default.
- W51931279 cites W2093120959 @default.
- W51931279 cites W2093975740 @default.
- W51931279 cites W2096576290 @default.
- W51931279 cites W2096802570 @default.