کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
1934765 | 1050650 | 2008 | 6 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Mild processing defect of porcine ÎF508-CFTR suggests that ÎF508 pigs may not develop cystic fibrosis disease
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
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.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochemical and Biophysical Research Communications - Volume 373, Issue 1, 15 August 2008, Pages 113-118
Journal: Biochemical and Biophysical Research Communications - Volume 373, Issue 1, 15 August 2008, Pages 113-118
نویسندگان
Yanli Liu, Ying Wang, Yong Jiang, Na Zhu, Haitao Liang, Lina Xu, Xuechao Feng, Hong Yang, Tonghui Ma,