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Fig. 4 | BMC Biology

Fig. 4

From: A human iPSC-derived inducible neuronal model of Niemann-Pick disease, type C1

Fig. 4

Axonal transport of acidic vesicles is impaired in NPC1−/− i3Neurons and can be improved with HPβCD treatment. A Kymograph analysis of acidic vesicle movement in axons of NPC1+/+ and NPC1−/− i3Neurons shows decreased movement in NPC1 mutant neurons. Images are represented in inverted grayscale. Lines with negative or positive slopes in the kymographs correspond to lysosomes moving in the anterograde or retrograde direction, respectively. Vertical lines correspond to static lysosomes. Quantification of percent and direction of moving acidic vesicles (B), speed of movement (C), and run length (D). All three parameters are impaired in axons of NPC1−/− i3Neurons and improve by treatment with 100 μM HPβCD (CD). E The average intensity of single lysosomes in the axon showed no significant differences. Values are the mean ± SD from > 100 vesicles in six neurons per condition. *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001 by non-parametric one-way ANOVA

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