Journal: Science Advances
Article Title: Lysosomal damage is a therapeutic target in Duchenne muscular dystrophy
doi: 10.1126/sciadv.adv6805
Figure Lengend Snippet: ( A ) Study setup. Dmd mdx-4Cv mice were started on TR treatment (2% dilution in drinking water) at 3 weeks. For groups 4 and 5, mice were injected intravenously with a rAAV9 encapsulating the μDys sequence at a dose of 7 × 10 12 vg/kg. ( B ) Seric CK analysis before the escape test (average ± SD, n = 5). ( C ) ELISA quantification of MYOM3 before the escape test (average ± SD, n = 5). ( D ) Global force evaluation (normalized to mice body weight, N/g) by the escape test (average ± SD, n = 5). ( E ) Histological characterization of muscles. Top panel: H&E labeling of GA muscle cross sections. Middle panel: Sirius red labeling of diaphragm and GA muscles. Bottom panel: Representative images of GA muscle serial cross sections immunostained for mouse immunoglobulin (IgG), laminin, and CD11b. Scale bars, 200 μm (H&E) and 500 and 100 μm (zoomed-in images). ( F ) Analysis of myofiber size distribution in GA muscles represented by the variance coefficient calculated as [SD of the muscle fiber size/mean of muscle fiber size] * 1000 (median with min-max values). ( G ) Centronucleation index in the GA muscle (median with min-max values). ( H and I ) Fibrosis analysis of GA and diaphragm by the quantification of Sirius red–positive areas on transversal sections (average ± SD). ( J ) Quantification of IgG uptake by myofibers (positive myofiber number normalized to muscle cross-sectional area). ( K ) Evaluation of CD11b + cell infiltration in the muscle by the quantification of CD11b area. An ANOVA test with Tukey’s multiple comparisons test was used for statistical comparisons (compared to WT control and mdx control). ANOVA: * P < 0.05, ** P < 0.05, *** P < 0.001, and **** P < 0.0001. Black asterisks represent relative comparison to the “WT control” group, and red asterisks represent comparison to the “mdx control” group. An unpaired two-tailed t test was used for statistical comparisons between mdx-μDys and mdx_μDys + TR groups. t test: * P < 0.05 and ** P < 0.01.
Article Snippet: An anti-MYOM3 polyclonal capture antibody (Proteintech Lab, ref. 17692-1-AP) was coated to the wells of a multiarray plate containing electrodes (MSD, ref. L15XA-3) (overnight, 4°C).
Techniques: Injection, Sequencing, Enzyme-linked Immunosorbent Assay, Muscles, Labeling, Control, Comparison, Two Tailed Test
Bio-Rad is a verified supplier 
![( A ) Study setup. Dmd mdx-4Cv mice were started on TR treatment (2% dilution in drinking water) at 3 weeks. For groups 4 and 5, mice were injected intravenously with a rAAV9 encapsulating the μDys sequence at a dose of 7 × 10 12 vg/kg. ( B ) Seric CK analysis before the escape test (average ± SD, n = 5). ( C ) ELISA quantification of MYOM3 before the escape test (average ± SD, n = 5). ( D ) Global force evaluation (normalized to mice body weight, N/g) by the escape test (average ± SD, n = 5). ( E ) Histological characterization of muscles. Top panel: H&E labeling of GA muscle cross sections. Middle panel: Sirius red labeling of diaphragm and GA muscles. Bottom panel: Representative images of GA muscle serial cross sections immunostained for mouse immunoglobulin (IgG), laminin, and CD11b. Scale bars, 200 μm (H&E) and 500 and 100 μm (zoomed-in images). ( F ) Analysis of myofiber size distribution in GA muscles represented by the variance coefficient calculated as [SD of the muscle fiber size/mean of muscle fiber size] * 1000 (median with min-max values). ( G ) Centronucleation index in the GA muscle (median with min-max values). ( H and I ) Fibrosis analysis of GA and diaphragm by the quantification of Sirius red–positive areas on transversal sections (average ± SD). ( J ) Quantification of IgG uptake by myofibers (positive myofiber number normalized to muscle cross-sectional area). ( K ) Evaluation of CD11b + cell infiltration in the muscle by the quantification of CD11b area. An ANOVA test with Tukey’s multiple comparisons test was used for statistical comparisons (compared to WT control and mdx control). ANOVA: * P < 0.05, ** P < 0.05, *** P < 0.001, and **** P < 0.0001. Black asterisks represent relative comparison to the “WT control” group, and red asterisks represent comparison to the “mdx control” group. An unpaired two-tailed t test was used for statistical comparisons between mdx-μDys and mdx_μDys + TR groups. t test: * P < 0.05 and ** P < 0.01.](https://pub-med-central-images-cdn.bioz.com/pub_med_central_ids_ending_with_2950/pmc12542950/pmc12542950__sciadv.adv6805-f9.jpg)