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. 2012 Sep 11;20(11):2120–2133. doi: 10.1038/mt.2012.181

Figure 8.

Figure 8

Lasting effect of AAV1-U7E6E8 in golden retriever muscular dystrophy dog (GRMD) after forelimb locoregional delivery. Dystrophin immunostaining (NCL-DYS2) in transverse sections of muscles: (a) flexor carpi ulnaris (FCU), and (b) extensor digitorum communis (EDC), 2 months after high pressure intravenous delivery of adeno-associated virus (AAV) vectors into the forelimb. Amounts of rescued dystrophin were variable from one biopsy to another, sometimes: dystrophin-positive fibers were scattered throughout the biopsy (a), while a biopsy could show large domains fully transduced next to negative areas (b). (c) Percentage of dystrophin-positive fibers found in muscles biopsies taken at different time points. (d) Quantification of the AAV genome copy number in biopsies at different time points. (e) Western blot of total protein (100 µg) extracted from biopsies at different time points stained with NCL-DYS2. Arrow indicates the full-length dystrophin as detected in samples from normal muscle used for comparison (25, 12, and 6%—diluted in GRMD to keep up total protein content). Samples were also hybridized with an actin-antibody to validate protein loadings.