Five months
of cemdomespib therapy maintained neuromuscular function
in three animal models of CMT1X. Mice were treated with 0.3 mg/kg
(A) or 3 mg/kg (B) cemdomespib beginning at 4 months of age (week
0) and grip strength measured biweekly. (A) *, p <
0.0002 versus time-matched T55I-Cx32def vehicle. ^, p < 0.003 versus time-matched Cx32def vehicle. Data are mean ±
SD and were analyzed using a two-way ANOVA and Sidak’s test.
Data were pooled from two independent cohorts of Cx32def and T55I-Cx32def
mice. (B) *, p < 0.001 versus time-matched vehicle.
Data are mean ± SD and were analyzed using a mixed-effect analysis
and Tukey’s test. (C) Five months of cemdomespib treatment
differentially improved grip strength between the genotypes. Red lines, p < 0.0001 versus respective genotype control; blue comparisons, p < 0.0001 versus 3 mg/kg Cx32def; purple comparison p < 0.003 versus 3 mg/kg T55I-Cx32def; *, p < 0.0001 versus 0.3 mg/kg T55I-Cx32def. Data for all groups are
mean ± SD. Symbols indicate the response of individual animals
in each group.