Figure 6.
DMD muscle phenotype can be rescued with Furin inhibition. A) dys-1; hlh-1 mutant animals were synchronized from the L1 stage and grown to young adulthood at 15°C (permissive temperature). Adult animals were then transferred to fresh NGM plates containing 1 µM Furin inhibitor or 10 µM MMP inhibitor or unc-68 RNAi plates and grown at 25°C (restrictive temperature) for 48 h. There was a significant decrease in the occurrence of muscle damage in dys-1; hlh-1 animals when treated with 1 µM Furin inhibitor, 10 µM MMP inhibitor, and unc-68 RNAi. B) Representative image of a muscle cell of dys-1; hlh-1 animals in the absence and presence of a Furin inhibitor. Number of muscle cell images/condition (n = 152, 109, 217, and 99 from left to right). Here, control animals are DMD animals treated with DMSO. Cont., control; inh., inhibitor. Scale bars, 10 µM. Letters (a–c) on top of bars indicate statistical significance; X, median value.