Absence of typical unfolded protein response in primary cultured cystic fibrosis airway epithelial cells.
Biochemical and Biophysical Research Communications(2006)
摘要
We examined whether the unfolded protein response is activated in cells expressing incorrectly folded cystic fibrosis transmembrane conductance regulator. Airway epithelial cells from three control and three CF patients homozygous for the ΔF508 mutation were tested. There were no differences in protein expression of the pro-apoptotic factor C/EBP homologous protein (CHOP) or the endoplasmic reticulum (ER) chaperone binding Ig protein. Nor were there differences in phosphorylation of protein kinase R-like ER kinase or eukaryotic initiation factor-2α, or the splicing of X-box binding protein (XBP)-1. However, CF cells showed increased mRNA expression of CHOP and XBP-1. A proteasome inhibitor increased CHOP expression in CF cells, suggesting that enhanced proteasome activation is responsible for the observed post-transcriptional regulation. Finally, CF cells were resistant to apoptosis, suggesting that post-transcriptional regulation of CHOP prevents apoptosis. While CHOP and XBP-1 mRNA expression is increased in CF cells, the classic UPR is not present.
更多查看译文
关键词
ATF6,BiP,CHOP,Endoplasmic reticulum,GADD153,PERK,XBP1
AI 理解论文
溯源树
样例
![](https://originalfileserver.aminer.cn/sys/aminer/pubs/mrt_preview.jpeg)
生成溯源树,研究论文发展脉络
Chat Paper
正在生成论文摘要