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Fig. 6 | Skeletal Muscle

Fig. 6

From: Mesoangioblast delivery of miniagrin ameliorates murine model of merosin-deficient congenital muscular dystrophy type 1A

Fig. 6

Miniagrin treatment restores expression of β1 integrin receptors. a Cryosections of the tibialis anterior muscle from dy2J mice untreated (saline solution) or treated with MABs + mMAG double stained with anti-myc (recognizing mMAG) and anti-β1 integrin antibody. The immunofluorescence shows that myc staining is present only in treated muscle. Staining for β1 integrin is more intense and diffuse in treated muscle. Images are acquired by confocal microscope with the same z-stack and laser intensity. DAPI staining identifies nuclei. b Western blot analysis of the tibialis anterior muscle from dy2J mice untreated or treated with MABs + mMAG or MABs alone and stained with anti β1 integrin, and β-actin as loading control. Quantification of Western blot analysis is reported as an average of three independent experiments and represented as the ratios β1 integrin/actin, assigning wild type as 1. A significant decrease of β1 integrin (n = 3) is present in muscle of dy2J mice as compare to wild type control; the amount of β1 integrin is significantly increased after treatment with MABs + mMAG. c Cryosections of wild type (Wt), dy2J untreated (saline), dy2J treated with MABs, and dy2J treated with MABs + mMAG stained with anti α7 integrin antibody and DAPI. Staining for α7 integrin was reduced in dy2J mice as compared to Wt and moderately increased in dy2J mice treated with MABs + mMAG. d Western blot analysis confirmed the decrease of α7 integrin in dy2J, whereas it was more similar to Wt in dy2J mice treated with MABs or MABs + mMAG; differences were not significant. e Western blot analysis of α5 integrin showed a significant increase in all the dy2J mice (treated or not treated) as compared to Wt controls. Scale bar = 50 μm in (a) and 100 μm in (c). Student t test; *p < 0.05; **p < 0.01; error bars indicate SEM

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