Ontology type: schema:ScholarlyArticle
1993-04
AUTHORSC Li, M Ramjeesingh, E Reyes, T Jensen, X Chang, J M Rommens, C E Bear
ABSTRACTThe cystic fibrosis transmembrane conductance regulator (CFTR) is a phosphorylation-regulated Cl- channel. In most mammalian cells, the functional consequences of the most common CF mutation, delta F508-CFTR, cannot be assessed as the mutant protein undergoes biosynthetic arrest. However, function can be studied in the baculovirus-insect cell expression system where delta F508-CFTR does not appear to undergo such arrest. Our results show that phosphorylation-regulated Cl- channel activity of delta F508-CFTR is similar to that of wild-type CFTR. This observation was confirmed in comparative studies of purified delta F508-CFTR and CFTR reconstituted in planar lipid bilayers. Therefore, we suggest that this common mutation does not result in a significant alteration in CFTR function. More... »
PAGES311-316
http://scigraph.springernature.com/pub.10.1038/ng0493-311
DOIhttp://dx.doi.org/10.1038/ng0493-311
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