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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: Impaired lipid metabolism in astrocytes underlies degeneration of cortical projection neurons in hereditary spastic paraplegia

Fig. 2

Impaired axonal transport of synaptophysin in ATL1 mutant cortical PNs derived from isogenic hPSCs. a, b Kymographs representing synaptic vesicles (SVs) transport along axons of WT, ATL1-P342S, ATL1-342-Cor, H9, ATL1-A161P #70 and ATL1-A161P #4 cortical PNs infected with synaptophysin::RFP virus. The x-axis indicates axon length from proximal to distal areas. The y-axis indicates time-lapse duration in min (y = 5 min). Scale bar: 5 µm. c Anterograde and d retrograde average moving velocities of SVs along axons in WT, ATL1-P342S, and ATL1-342-Cor cortical PNs. e Anterograde and f retrograde average moving velocities of SVs along axons in H9, ATL1-A161P #70, and ATL1-A161P #4 cortical PNs. g, h Graphs show the ratios of moving SVs to total SVs (g) and moving SVs per 100 µm of axon (h) in WT, ATL1-P342S, and ATL1-342-Cor cortical PNs. k, l Graphs represent the ratio of moving SVs in relation to the total number of SVs (k) and the ratio of moving SVs per 100 µm of axon (l) in H9, ATL1-A161P #70 and ATL1-A161P #4 cortical PNs. i, j Number of total SVs per 100 µm of axon (i) and the ratio of anterograde moving SVs in relation to moving SVs (j) in WT, ATL1-P342S and ATL1-342-Cor cortical PNs. m, n Number of total SVs per 100 µm of axon (m) and the ratio of anterograde moving SVs in relation to moving SVs (n) in H9, ATL1-A161P #70 and ATL1-A161P #4 cortical PNs. Data are represented as mean ± SEM from 3 independent samples. The statistical significance of mean values among three groups was analyzed using ANOVA. *p < 0.05 compared to WT (for ATL1-P342S) and H9 (for ATL1-A161P #70 and #4), respectively. #p < 0.05 compared to ATL1-P342S

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