Figure 1: Analysis of cMD1 expression by immunostaining and western-blot in muscles of GRMD dogs injected with rAAV2/8-Spc512-cMD1 by the LR route.
From: Long-term microdystrophin gene therapy is effective in a canine model of Duchenne muscular dystrophy
(a) Dystrophin immunostaining (NCL-DYSB) on transverse sections of muscle samples. Representative results are presented for healthy (WT) and untreated GRMD dogs and for four different muscles of dog LR1 sampled at the time of killing (non injected and injected forelimb, below and above the tourniquet). Scale bar, 100 μm. (b) Western-blot analysis of total proteins (50 μg) extracted from muscles samples. Representative results for the same muscles as in a are shown. GRMD myoblasts transduced with the rAAV2/8-Spc5.12-cMD1 vector were used as positive control. The blot was stained with MANEX-1011C to reveal the presence of the 427 kDa dystrophin protein (WT dog) and the 138 kDa cMD1 protein, with an anti-GAPDH antibody as a loading control. The level of cMD1-positive fibres detected by immunostaining from the same muscle samples are indicated under each panel.
