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. 2017 Sep 14;26(23):4668–4679. doi: 10.1093/hmg/ddx348

Figure 1.

Figure 1.

ALS mutant SOD1 impairs axonal transport of mitochondria. Axonal transport of mitochondria was analyzed in motor neurons (A) and cortical neurons (B). (Aa, Ba) Kymographs show transport of mitochondria in rat motor neurons and cortical neurons expressing EGFP (Ctrl), EGFP-SOD1 WT, A4V, G37R or G93A. (Ab, c; Bb, c) Quantitative analysis of mitochondrial transport shows that expression of ALS mutant SOD1 significantly impairs overall motility of mitochondria (Ab, Bb—Motile) because of a selective block of anterograde (Ab, Bb—Anterograde), but not retrograde (Ab, Bb—Retrograde) transport. As a consequence, SOD1 G93A disturbed the balance of transport to promote net retrograde movement (Ac, Bc). Results are shown as mean ± SEM, statistical significance was determined by one-way ANOVA followed by Fisher’s LSD test, ns, not significant, * P < 0.05, **** P < 0.0001, N (cortical neurons): Ctrl: 24, WT: 21, G93A: 24 from 5 experiments; N (motor neurons) = Ctrl: 16, WT: 20, A4V: 20, G37R: 21, G93A: 21 from 3 experiments.