Fig. 3: Proteomic and inflammatory features of dsyferlinopathic skeletal muscle mice are modulated following ONX-0914 treatment. | Cell Death & Disease

Fig. 3: Proteomic and inflammatory features of dsyferlinopathic skeletal muscle mice are modulated following ONX-0914 treatment.

From: Inhibition of the immunoproteasome modulates innate immunity to ameliorate muscle pathology of dysferlin-deficient BlAJ mice

Fig. 3

Proteomic analysis of IP subunits (A); alarmins and inflammatory mediators (PTX3 and GPx1) (B); MAPK kinases and AKT1/2/3 and their phosphorylated isoforms (C) in psoas of 6 m BlAJ, 12 m BlAJ and BlAJ+ONX, 12 m C57Bl mice. Psoas stained with isolectin (in green), PTX3 (in magenta) with nuclei stained in DAPI (blu) (scale bar: 10 μm) and its magnification (scale bar: 10 μm) with white arrows indicating cells co-expressing PTX3 and isolectin. The histogram represents the counting of PTX3 fluorescence (D). Immunohistological staining of skeletal muscles for Iba1 (in red) and CD206 (in green). Nuclei were counterstained with DAPI (blue). Scale bar: 50 μm. Quantification of macrophage percentages. Mac1 were stained with Iba1+ and Mac2 with CD206+ in 6 m BIAJ, 12 m BlAJ and BlAJ+ONX, 12 m C57Bl skeletal muscles (E). Data are presented as mean ± SD of n = 3 independent experiments with n = 3–6 animals/group. One-way ANOVA, Tukey multiple comparison test for WB and non-parametric test followed by Kruskal–Wallis test for PTX3+ cells’ and macrophages’ counting: *p < 0.05, **p < 0.01, ***p < 0.001 and ****p < 0.0001.

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