5'-adenosine monophosphate mediated cooling treatment enhances ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) stability in vivo
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- 作者：Yueqiang Zhang ; William G. O'Brien III ; Zhaoyang Zhao…
- 关键词：Misfolded protein ; Mutant ; Genetic disorder ; Temperature ; sensitive ; Whole body cooling ; Rescue ; Treatment ; Hypothermia ; Hypometabolism ; Cystic Fibrosis
- 刊名：Journal of Biomedical Science
- 出版年：2015
- 出版时间：December 2015
- 年：2015
- 卷：22
- 期：1
- 全文大小：2,120 KB
- 刊物类别：Biomedical and Life Sciences
- 刊物主题：Biomedicine
Biomedicine - 出版者：Springer Netherlands
- ISSN：1423-0127
文摘
Background Gene mutations that produce misprocessed proteins are linked to many human disorders. Interestingly, some misprocessed proteins retained their biological function when stabilized by low temperature treatment of cultured cells in vitro. Here we investigate whether low temperature treatment in vivo can rescue misfolded proteins by applying 5’-AMP mediated whole body cooling to a Cystic Fibrosis (CF) mouse model carrying a mutant cystic fibrosis transmembrane conductance regulator (CFTR) with a deletion of the phenylalanine residue in position 508 (ΔF508-CFTR). Low temperature treatment of cultured cells was previously shown to be able to alleviate the processing defect of ΔF508-CFTR, enhancing its plasma membrane localization and its function in mediating chloride ion transport.