Fig. 1: JP18 B6 and JP18/JY13 B6 mice are representative models for CMT1A pathology.

a Representative western blot gel showing the Pmp22 protein content in sciatic nerve normalized to tubulin then, reported on WT and the corresponding protein quantification. b Time taken by the mice to perform the beam walking, the locotronic test and the force of their total limbs in gram. c Analysis of the electrophysiological tests CMAP and NCV. d Semi-thin section of sciatic nerves from WT B6, JP18 B6, and JP18/JY13 B6 mice scanned at 40×. Scale bar 20 µm. Fiber count quantification was done using image J software at 70×. To determine the cutoff between small and large fibers a quadratic model analysis was performed. The cutoff is: 7.4 with a 95% CI at [6.95–7.85] for WT (blue line), 7.20 CI [6.39–7.99] for JP18 (dark dashed) and 6.35 CI [5.54–7.10] for JP18/JY13 (pink line). e Transmission electron microscopy (TEM) images (5kx) of ultrathin sciatic nerve sections of the tested mice groups and the g-ratios analysis of myelinated fibers. Scale bar 2 µm. High magnification images (120kx) were taken to show the inter myelin layer distance which is represented by yellow lines for WT and red lines for JP18 and JP18/JY13 group followed by interperiodic analysis graph. Scale bar 50 nm. All the experiments were done on six mice per group. Data represent predicted means with 95% confidence intervals for layer distance while mean ± s.e.m. was represented elsewhere. *p < 0.05; **p < 0.01; ***p < 0.001 using ANOVA analysis followed by Tukey’s multiple comparison tests.