Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.
Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.
复制标题
修复 deltaF508 CFTR 折叠和细胞靶向缺陷的治疗方法。
DOI：
10.1016/s0169-409x(02)00148-5
复制
发表时间：
2002
影响因子：
16.1
通讯作者：
P. Zeitlin
中科院分区：
文献类型：
--
作者：
K. Powell;P. Zeitlin
囊性纤维化跨膜调节因子（CFTR）基因中的ΔF508突变是CF中最常见的突变。突变CFTR蛋白在多个功能方面有缺陷，包括cAMP调节的氯离子电导、核苷酸转运和对其他离子通道的调节作用。由于ΔF508蛋白在内质网（ER）中的翻译和折叠过程中也是温度敏感和不稳定的，因此大多数新生链都是ER过早蛋白水解的目标。本文着重于事件发生在ER折叠过程中，并审查潜在的治疗干预的目标。
The ΔF508 mutation in the cystic fibrosis transmembrane regulator (CFTR) gene is the most common mutation in CF. The mutant CFTR protein is defective with respect to multiple functions including cAMP-regulated chloride conductance, nucleotide transport, and regulatory actions on other ion channels. Since the ΔF508 protein is also temperature-sensitive and unstable during translation and folding in the endoplasmic reticulum (ER), most of the nascent chains are targeted for premature proteolysis from the ER. This paper focuses on the events that occur in the ER during folding and reviews potential targets for therapeutic intervention.
CFTR（囊性纤维化跨膜电导调节因子）在食管鳞癌中的表达及作用
DOI：
--
发表时间：
2020
期刊：
影响因子：
--
作者：
松本順久;塩﨑敦;工藤道弘;小菅敏幸;小西博貴;窪田健;藤原斉;岡本和真;岸本光夫;大辻英吾
通讯作者：
大辻英吾