Fig. 4

Functional motor unit and behavioral deficits in CHCHD10^R15L and CHCHD10^S59L transgenic mice. a Schematic of in vivo motor unit recording. Stimulating electrodes placed at the sciatic nerve and recording electrodes placed at the gastrocnemius muscle with grounding electrode at the tail. b A representative CMAP waveform from a 10-month-old wild type mouse. Stimulus intensity at the sciatic nerve was increased at increments of 0.025 mA until compound muscle action potential (CMAP) was evoked in an all-or-none fashion and repeated for a total of 10 increments to calculate single motor unit potential (SMUP) and motor unit number estimation (MUNE). c Motor unit number estimation (MUNE) of 10-month-old WT, CHCHD10^WT, CHCHD10^R15L, and CHCHD10^S59L mice (1-way ANOVA, F(2, 20) = 36, P < 0.0001, posthoc Tukey, ##P < 0.0001, n = 6–10 mice/genotype). d Sciatic nerve CMAP velocity (m/s) measured between the stimulating and recording electrodes in 10-month-old WT, CHCHD10^WT, CHCHD10^R15L, and CHCHD10^S59L mice (1-way ANOVA, F(2, 22) = 91.14, P < 0.0001, posthoc Tukey, ##P < 0.0001, n = 6–12 mice/genotype). e Grip strength (gram force) in 10-month-old WT, CHCHD10^WT, CHCHD10^R15L, and CHCHD10^S59L mice (1-way ANOVA, F(3, 58) = 9.072, P < 0.0001, posthoc Tukey, **P < 0.01, #P < 0.001, n = 8–23 mice/genotype). f Rotarod test measured in latency to fall (sec) in 10-month-old WT, CHCHD10^WT, CHCHD10^R15L, and CHCHD10^S59L mice (1-way ANOVA, F(3, 50) = 12, P < 0.0001, posthoc Tukey, *P < 0.05, ##P < 0.0001, n = 8–19 mice/genotype)