Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutations
详细信息 查看全文
- 作者：Sandipan Datta sddatta@ucdavis.edu" class="auth_mail" title="E-mail the corresponding author ; Alexey Tomilov atomilov@ucdavis.edu" class="auth_mail" title="E-mail the corresponding author ; Gino Cortopassi ; gcortopassi@ucdavis.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：LHON ; Mitochondria ; High-throughput screening ; Complex I
- 刊名：Mitochondrion
- 出版年：2016
- 出版时间：September 2016
- 年：2016
- 卷：30
- 期：Complete
- 页码：177-186
- 全文大小：1116 K
文摘
- •
LHON primary mutations sensitize ATP synthesis to complex I inhibitor rotenone.
- •
A high-throughput ATP synthesis-based screen was developed for LHON drug discovery.
- •
A library of 1600 clinically used drugs was screened for repurposing.
- •
Papaverine and zolpidem individually can rescue the rotenone sensitivity in LHON mutants.
- •
The combination of the drugs shows an additive effect on ATP synthesis rescue.