FIGURE 5.

Axon degeneration under oxidative stress increased in SPAST patients and is rescued by tubulin-binding drugs. (A,B) Untreated control and patient axons. Control and patient axons exposed to 20 μM H₂O₂ for 3 h (C,D) and 6 h (E,F). Patient axons treated with epothilone D (G) or noscapine (H) for 24 h before exposing to 20 μM H₂O₂ for 3 h. Scale bar = 25 μm. I: Degeneration index (DI) at different periods of treatment H₂O₂ exposure. Patient DIs (filled symbols) were significantly different from control DIs (open symbols) at 3 and 6 h. (J) DIs of untreated controls (open symbols) and untreated patients (filled symbols) and patient cultures treated with epothilone D or noscapine for 24 h prior to 3 h exposure to 20 μM H₂O₂. Statistics shown for differences between untreated patient axons and other groups.