Figure 2.

Expression of mAAV8-Jazz and utrophin up-regulation in mdx mice. a: Evaluation of Jazz-mRNA expression by RT-PCR of skeletal muscle mRNA from AAV8-Jazz-treated and untreated mdx mice. A β2M control from the same samples is shown below. b: Evaluation of Jazz protein expression by Western blot analysis in skeletal and cardiac muscles and in liver tissues. Five-day-old mdx mice were intraperitoneally injected and examined 15 days after injection using the anti-myc tag monoclonal 9E10 antibody. Detection of α-tubulin was used to normalize the amount of proteins. c: Immunohistochemistry of the quadriceps muscle derived from 2-month-old untreated and mAAV8-Jazz-treated mdx mice stained with the anti-myc tag monoclonal 9E10 antibody (red). The extracellular matrix is stained with the anti-laminin polyclonal antibody (green), and nuclei are counterstained with Dapi. Scale bar: 20 μm. d: Quantification by real-time PCR of utrophin transcripts from skeletal and heart muscles isolated from 2-month-old untreated mdx mice and mAAV8-EGFP-treated or mAAV8-Jazz-treated mdx mice. The gene expression ratio between utrophin and β2-microglobulin (β2 M) is shown as the mean ± SEM. from three independent experiments performed in triplicate. *P < 0.05 and **P < 0.01 indicate statistical significance by t-test. e: Western blot analysis of utrophin protein levels in abdominal, heart, and quadriceps muscles isolated from 2-month-old mAAV8-Jazz-treated or mAAV8-EGFP-treated mdx mice. Representative individual mice are indicated with numbers. Detection of laminin was used to normalize the amount of proteins.