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Fig. 4 | Molecular Medicine

Fig. 4

From: Dystrophin R16/17 protein therapy restores sarcolemmal nNOS in trans and improves muscle perfusion and function

Fig. 4

Sarcolemmal nNOS is successfully restored by systemic delivery of mTAT.R16/17.GFP.Pal protein in ΔR4 mice. a After systemic delivery of mTAT.R16/17.GFP.Pal protein in ΔR4 mice, dystrophin (Dys), syntrophin (Syn) and nNOS were examined on serial sections of the TA muscle. The GFP signal confirmed that mTAT.R16/17.GFP.Pal was transferred to the TA muscle. With R16/17 protein transfer, sarcolemmal nNOS was successfully restored, as shown by nNOS immunostaining and nNOS activity staining at the muscle membrane. Without R16/17 injection, despite the presence of ΔR4 micro-dystrophin and syntrophin at the sarcolemma, sarcolemmal nNOS was still absent. Asterisk: the same myofiber on serial sections. Scale bar = 50 μm. b The signal intensity of nNOS immunostaining was quantified and analyzed by ANOVA. After R16/17 protein transfer, compared to mdx4cv and non-injected ΔR4 mice, sarcolemmal nNOS in ΔR4 mice was statistically improved (** p < 0.0001). Sarcolemmal nNOS in the muscle of BL6 is significantly higher than mdx4cv, ΔR4 and ΔR4 + R16/17 (* p < 0.0001)

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Molecular Medicine

ISSN: 1528-3658