Fig. 8. VCP^P134L knock-in mutants cause VCP aggregation and functional degeneration.

a Localization of endogenously expressed VCP^WT-sfGFP and VCP^P134L-sfGFP in young and aged TTM muscles. b Quantification of puncta density for data in a. Data presented as mean and SEM. c Representative traces recorded from the DLM muscle in wild type and VCP^P134L. Failures are indicated by asterisks. Average failure rates are indicated below each trace (n = 10 animals for each genotype), stimulus artifact removed for clarity. d Quantification of data in c for the genotypes indicated. Stimulation was achieved either through activation of the giant fiber (a) or direct motoneuron stimulation (MN stim). e Lifespan of VCP^WT-sfGFP (n = 103) and VCP^P134L-sfGFP (n = 97). f Negative geotaxis assay for genotypes at indicated ages. Data from three independent experiments with 20 animals each are presented as mean and SEM for each age point. g Representative images of NMJs stained with Brp (green) and DLG (magenta). h Quantification of Brp per bouton. Data presented in a box and whisker plot (minimum, 1st quartile, median, 3rd quartile, and maximum). Scale bars indicated on images. See also Supplementary Fig. S7. ANOVA for multiple comparisons (*p < 0.05; **p < 0.01; ***p < 0.001).