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- W2080145794 abstract "Cystic fibrosis (CF) is a common genetic disease for which the gene was identified within the last decade. Pulmonary disease predominates in this ultimately fatal disease and current therapy only slows the progression. CF transmembrane regulator (CFTR), the gene product, is an integral membrane glycoprotein that normally functions as a chloride channel in epithelial cells. The most common mutation, ΔF508, results in mislocalization and altered glycosylation of CFTR. Altered fucosylation and sialylation are hallmarks of both membrane and secreted glycoproteins in CF and the focus here is on these investigations. Oligosaccharides from CF membrane glycoproteins have the Lewis x, selectin ligand in terminal positions. In addition, two major bacterial pathogens in CF, Pseudomonas aeruginosa and Haemophilus influenzae, have binding proteins, which recognize fucose in α1,3 linkage and asialoglycoconjugates. We speculate that the altered terminal glycosylation of airway epithelial glycoproteins in CF contributes to the chronic infection and robust inflammatory response in the CF lung. Understanding the effects of mutant CFTR on glycosylation may provide further insight into the regulation of glycoconjugate processing as well as therapy for CF." @default.
- W2080145794 created "2016-06-24" @default.
- W2080145794 creator A5037250656 @default.
- W2080145794 creator A5082629780 @default.
- W2080145794 date "1999-10-01" @default.
- W2080145794 modified "2023-10-16" @default.
- W2080145794 title "Terminal glycosylation in cystic fibrosis" @default.
- W2080145794 cites W1525551129 @default.
- W2080145794 cites W1529784093 @default.
- W2080145794 cites W1537652082 @default.
- W2080145794 cites W1547481148 @default.
- W2080145794 cites W1556308744 @default.
- W2080145794 cites W1576820849 @default.
- W2080145794 cites W1579581978 @default.
- W2080145794 cites W1592694746 @default.
- W2080145794 cites W1616824889 @default.
- W2080145794 cites W1909269101 @default.
- W2080145794 cites W1966912799 @default.
- W2080145794 cites W1968578988 @default.
- W2080145794 cites W1968774879 @default.
- W2080145794 cites W1969697265 @default.
- W2080145794 cites W1971932849 @default.
- W2080145794 cites W1972402373 @default.
- W2080145794 cites W1972415233 @default.
- W2080145794 cites W1975971473 @default.
- W2080145794 cites W1976248923 @default.
- W2080145794 cites W1981956842 @default.
- W2080145794 cites W1983611845 @default.
- W2080145794 cites W1983721737 @default.
- W2080145794 cites W1985157469 @default.
- W2080145794 cites W1987463930 @default.
- W2080145794 cites W1987760846 @default.
- W2080145794 cites W1988274034 @default.
- W2080145794 cites W1988868648 @default.
- W2080145794 cites W1989045338 @default.
- W2080145794 cites W1989721672 @default.
- W2080145794 cites W1990615889 @default.
- W2080145794 cites W1995240556 @default.
- W2080145794 cites W1996768081 @default.
- W2080145794 cites W1997311933 @default.
- W2080145794 cites W1997893339 @default.
- W2080145794 cites W1999619695 @default.
- W2080145794 cites W1999746307 @default.
- W2080145794 cites W2005450492 @default.
- W2080145794 cites W2013163864 @default.
- W2080145794 cites W2015072327 @default.
- W2080145794 cites W2015357935 @default.
- W2080145794 cites W2016409440 @default.
- W2080145794 cites W2019187279 @default.
- W2080145794 cites W2021167060 @default.
- W2080145794 cites W2026878065 @default.
- W2080145794 cites W2028171116 @default.
- W2080145794 cites W2032971663 @default.
- W2080145794 cites W2034951338 @default.
- W2080145794 cites W2035451636 @default.
- W2080145794 cites W2036774130 @default.
- W2080145794 cites W2037459969 @default.
- W2080145794 cites W2037702127 @default.
- W2080145794 cites W2039599894 @default.
- W2080145794 cites W2039601720 @default.
- W2080145794 cites W2041418712 @default.
- W2080145794 cites W2042843548 @default.
- W2080145794 cites W2043286012 @default.
- W2080145794 cites W2045803045 @default.
- W2080145794 cites W2047455643 @default.
- W2080145794 cites W2049827739 @default.
- W2080145794 cites W2052196004 @default.
- W2080145794 cites W2052588826 @default.
- W2080145794 cites W2052750543 @default.
- W2080145794 cites W2064007256 @default.
- W2080145794 cites W2070122091 @default.
- W2080145794 cites W2071546385 @default.
- W2080145794 cites W2072844460 @default.
- W2080145794 cites W2073544938 @default.
- W2080145794 cites W2073727172 @default.
- W2080145794 cites W2075390708 @default.
- W2080145794 cites W2077264898 @default.
- W2080145794 cites W2079234445 @default.
- W2080145794 cites W2080423498 @default.
- W2080145794 cites W2080461650 @default.
- W2080145794 cites W2082886826 @default.
- W2080145794 cites W2084491822 @default.
- W2080145794 cites W2088043549 @default.
- W2080145794 cites W2088456947 @default.
- W2080145794 cites W2089591893 @default.
- W2080145794 cites W2089986311 @default.
- W2080145794 cites W2090781939 @default.
- W2080145794 cites W2117143712 @default.
- W2080145794 cites W2125235151 @default.
- W2080145794 cites W2125391335 @default.
- W2080145794 cites W2137300408 @default.
- W2080145794 cites W2139221400 @default.
- W2080145794 cites W2139900748 @default.
- W2080145794 cites W2142318878 @default.
- W2080145794 cites W2147286631 @default.
- W2080145794 cites W2147839947 @default.
- W2080145794 cites W2151134543 @default.
- W2080145794 cites W2328538504 @default.