Figure 2.

Analysis of the Mpz^D61N mouse model. (A) WB for P0 on proteins extracted from sciatic nerves of WT and Mpz^D61N/+ animals treated with PNGaseF. Before PNGase digestion P0D61N/+ extracts show a double band; after treatment with PNGaseF, the MW of P0 in Mpz^D61N/+ samples decreases to a single band of the same MW as in the WT. (B) Table describing the tremor in WT and Mpz^D61N/+ mice at different time points; the scale is visual and goes from – (no tremor) to + (mild tremor) to ++++ (very severe tremor). (C) Rotarod analysis, performed at 3 months of age, shows that Mpz^D61N/+ have a severe reduction in motor capacity as compared with WT; ^****P-value < 0.0001 by two-way ANOVA with Sidak multiple comparison post-hoc test. n = 8 for each genotype. (D) Grip strength test, performed at 3 months of age, shows that Mpz^D61N/+ mice have reduced strength in hind limbs as compared with WT. ^*P-value = 0.0246 by unpaired Student t-test. n = 13 for each genotype.