Figure 8 | Scientific Reports

Figure 8

From: Substrate reduction therapy for Krabbe disease and metachromatic leukodystrophy using a novel ceramide galactosyltransferase inhibitor

Figure 8

Chronic CGT inhibition affects PNS and CNS function and integrity. Wild-type mice were treated with S202 by IP injection three times per week for 8 weeks starting on PND15. The ability of mice to hang from a wire mesh was evaluated weekly starting on week 2 of treatment (A). Sciatic NCVs were recorded from mice that had been treated with S202 (B). G-ratio analysis of sciatic nerve (C). H&E stained brain sections from S202 revealed vacuolation in S202 treated animals at either dose (E arrow) that was not seen in vehicle treated animals (D). Severity of vacuolation was scored by a trained pathologist on a scale of 1–4 in white matter (F) or gray matter (G) in brain and spinal cord. (n = 6–12).

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