Neuronal expression of USP14CA leads to decreased body weight, muscle weight, and motor performance. (A) Survival curves for wild type, TgUsp14CA, TgUsp14, and ax
J mice. TgUsp14 mice express wild type USP14 under the Thy1.2 promoter and serve as a control for neuronal USP14 overexpression in TgUsp14CA mice. ax
J mice have a 95% reduction of USP14 in all tissues. (B) Tail suspension assays show hind limb clasping in TgUsp14CA mice versus the typical splaying observed in 6-week-old wild type and TgUsp14 mice. Assays of (C) body mass, (D) muscle mass, (E) muscle strength and (F) motor performance of 2- to 24-week-old wild type, TgUsp14CA, and TgUsp14 mice. Muscle strength was assessed by a forelimb grip-force assay and motor performance was assessed by time required to traverse an elevated beam. All data are shown as mean ± SEM. Symbols represent Mann-Whitney tests compared against wild type and corrected for multiple comparisons with a Bonferonni adjustment where appropriate; *p < 0.05, **p < 0.01, ***p < 0.001; n = at least 4 animals per genotype per time point.