囊性纤维化F508del-CFTR突变患者CFTR功能恢复数据集

资源简介

囊性纤维化F508del-CFTR突变患者CFTR功能恢复数据集

【数据集背景】

本数据集源站标题为《Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation》。配套论文《Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation》发表于 Autophagy（2014），DOI：10.4161/15548627.2014.973737。源站首次发布：2015-06-19。

【数据内容】

源站原始描述：Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures rescue the functional expression of the most frequent pathogenic CFTR mutant, F508del, at the respiratory epithelial surface and reduce lung inflammation in CftrF508del homozygous mice. Cysteamine, the reduced form of cystamine, is an FDA-approv…

【数据结构与技术规格】

文件数 2 个，合计 227.85 KB。kaup_a_973737_sm9573.doc：application/msword，47.10 KB。kaup_a_973737_sm9572.pdf：application/pdf，180.75 KB。数据集 DOI：10.6084/m9.figshare.1247659.v5。

【主题与分类】

源站学科分类：Plant Biology、Infectious Diseases、Cancer、Developmental Biology、Immunology、Physiology。主题标签：Epithelial cells、CXCL 8 protein levels、BECN、CF patients、F 508del、plasma membrane expression、SQSTM 1 levels、F 508del protein、tnf、cysteamine。

【适用方向】

源站学科分类标注为 Plant Biology、Infectious Diseases、Cancer、Developmental Biology、Immunology、Physiology。

【获取与许可】

源站页面：https://tandf.figshare.com/articles/dataset/Restoration_of_CFTR_function_in_patients_with_cystic_fibrosis_carrying_the_F508del_CFTR_mutation/1247659

使用许可：CC BY 4.0（https://creativecommons.org/licenses/by/4.0/）。

规范引用：Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. Taylor & Francis. De Stefano, Daniela; R Villella, Valeria; Esposito, Speranza; Tosco, Antonella; Sepe, Angela; De Gregorio, Fabiola; et al

【使用注意】

· 源站最后修改于 2023-05-30，引用时请注意版本时效。