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Figure 5 | Orphanet Journal of Rare Diseases

Figure 5

From: Polytherapy with a combination of three repurposed drugs (PXT3003) down-regulates Pmp22 over-expression and improves myelination, axonal and functional parameters in models of CMT1A neuropathy

Figure 5

Daily oral treatment of CMT1A rats with PXT3003 improves myelination and electrophysiology. A 4-month PXT3003 treatment (BCL 30 μg/kg, NTX 3.5 μg/kg and SRB 1.05 mg/kg) of TG rats significantly increased the number of myelinated axons in sciatic nerve cross sections (A) mostly in the small to medium sized axon class of myelinated axons (< 4 μm) (B). n = 12, 12 and 15 animals for respectively WT vehicle, TG vehicle and TG PXT3003 groups. (C) 8-month PXT3003 treatment increased the motor nerve conduction velocity of TG rats in sciatic nerve. n = 11, 9 and 7 animals for respectively WT vehicle, TG vehicle and TG PXT3003 groups. (D) Representative images of toluidine blue-stained sciatic nerve cross sections for each group. Scale bar: 25 μm. *,+ P < 0.05; ** P < 0.01; ***P < 0.001 vs TG vehicle; ANOVA with Dunnett’s test (except in (C), + t-test). Data are shown as mean + SEM.

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