Figure 3.

Electron Microscopic Analyses of the CNS after Lonaprisan Therapy in PMD Mice

(A) Electron microscopic graphs of the corticospinal tract showed preservation of myelinated axons after Lonaprisan treatment.

(B) Myelinated axons are reduced in PMD mice compared to wild-type controls, and after Lonaprisan therapy myelinated axons are increased.

(C) Nonmyelinated axon number was increased in the disease situation but was unaltered by Lonaprisan treatment.

(D and E) In PMD mice, Lonaprisan therapy did not increase the mean myelin sheath thickness (D) and corresponding myelin sheath thickness according to axonal size (E).

(F and G) Plp1 mRNA expression levels correlated to the myelinated axon number (F), and myelinated axon number correlated to the phenotype (G).

Scale bars represent 1.7 μm. CST indicates corticospinal tract, ns indicates not significant, ^∗p < 0.05, ^∗∗∗p < 0.001, shown mean ± SEM.