Article
Hsp90 inhibition protects against inherited retinal degeneration.
Human molecular genetics - 15 Apr 2014
Aguilà Mònica, Bevilacqua Dalila, McCulley Caroline, Schwarz Nele, Athanasiou Dimitra, Kanuga Naheed, Novoselov Sergey S, Lange Clemens A K, Ali Robin R, Bainbridge James W, Gias Carlos, Coffey Peter J, Garriga Pere, Cheetham Michael E
Abstract excerpt
The molecular chaperone Hsp90 is important for the functional maturation of many client proteins, and inhibitors are in clinical trials for multiple indications in cancer. Hsp90 inhibition activates the heat shock response and can improve viability in a cell model of the P23H misfolding mutation in rhodopsin that causes autosomal dominant retinitis pigmentosa (adRP). Here, we show that a single low dose of the...
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