Fig. 4
From: VCP/p97 inhibitor CB-5083 modulates muscle pathology in a mouse model of VCP inclusion body myopathy
Biomarker analysis of VCPR155H/R155H mice treated with CB-5083. A Quadriceps lysates of WT, and vehicle and CB-5083-treated VCPR155H/R155H mice (n = 3–5 in each group) were subjected to Western blot analysis and immunoblotted with antibodies against major disease biomarkers including MFN2, p62, TFEB, and TDP-43. GAPDH was used as a loading control. p62, TFEB, and TDP-43 levels were diminished in VCPR155H/R155H mice upon CB-5083 treatment. B Western blots of quadriceps lysates, immunoblotted with antibody against TDP-43 and phosphorylated TDP-43 (p-TDP-43). P-TDP-43 was diminished in VCPR155H/R155H muscles upon CB-5083 treatment. C Rotarod analysis for VCPR155H/R155H mice treated with CB-5083 (n = 8) compared to vehicle treatment (n = 7) showed differences, however were not significant, D Densitometric analysis of Western blots shown in (A, B). Western blot was repeated three times. Statistical analysis was performed by one-way ANOVA followed by Fisher’s LSD test *P < 0.05