Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
1934765 | Biochemical and Biophysical Research Communications | 2008 | 6 Pages |
Abstract
Recent efforts have made significant progress in generating transgenic pigs with the ÎF508-CFTR mutation to model the lung and pancreatic disease of human cystic fibrosis. However, species differences in the processing and function of human, pig and mouse ÎF508-CFTR reported recently raise concerns about the phenotypic consequence of the gene-targeted pig model. The purpose of the present study was to characterize the ÎF508 mutant of porcine CFTR to evaluate the severity of its processing defect. Biochemical and immunofluorescence analysis in transfected COS7 and FRT cells indicated that pig ÎF508-CFTR efficiently targets to the plasma membrane and is present mainly as the mature glycosylated protein. Functional characterization in stably transfected FRT cells by fluorometric and electrophysiological assays supported efficient plasma membrane targeting of pig ÎF508-CFTR. The mild cellular processing defect of pig ÎF508-CFTR suggests that its gene-targeted pig model may not develop the lung and pancreatic phenotypes seen in CF patients.
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Authors
Yanli Liu, Ying Wang, Yong Jiang, Na Zhu, Haitao Liang, Lina Xu, Xuechao Feng, Hong Yang, Tonghui Ma,