Figure 8. ATM protein expression is restored after read-through compound exposure in explant tissues from Atm^R35X/R35X and Atm^Q35X/Q35X mice.

Spleen and cerebellar explant tissue from Atm^R35X/R35X and Atm^Q35X/Q35X mice were treated with vehicle, the read-through compounds G418 (100 µM) or GJ103 (100 µM) for 72 hr. ATM immunoblots show recovery of ATM (MW 350 kDa) production in both the spleen and cerebellum. Equal loading was assessed via housekeeping genes (Actin or GAPDH) and ponceau staining. ATM, Ataxia Telangiectasia mutated.