Figure 4.

Pharmacological rescue of neuronal and mitochondrial phenotypes in HSP-SPG7 patient neurons. (A) Shows the Bz-423 drug treatment timeline. (B–D) Live untreated control, untreated patient and Bz-423 treated patient neurons labelled with calcein – to identify cells, TMRM – to identify mitochondrial and hoechst – to identify nucleus. (E–G) Neurites were segmented and identified using automated image analysis in untreated control, FIGURE 4 (Continued)untreated patient and Bz-423 treated patient neurons. (H–L) Multiple parameters of neurite morphology were analyzed in untreated control, untreated patient and Bz-423 treated patient neurons. These parameters included neurite (H) length, (I) roots, (J) branching, (K) segments and (L) extremities. (M–O) Multiple parameters of mitochondrial morphology and membrane potential were analyzed in untreated control, untreated patient and Bz-423 treated patient neurons. These parameters included mitochondrial (M) area, (N) perimeter and (O) length and (P) TMRM intensity. (Q–R) Viability and axonal degeneration of untreated control, untreated patient and Bz-423 treated patient neurons. Data is presented as Mean ± SEM. Scale bar: 100 μm.