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Fig. 4 | Orphanet Journal of Rare Diseases

Fig. 4

From: Neural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses

Fig. 4

Hydroxypropyl-β-cyclodextrin (HPBCD) ameliorated enlarged lysosomes in NCL NSCs. a Representative image of HPBCD’s effect on reducing LysoTracker staining in NCL NSCs. The maximum reduction of enlarged lysosomes ranged from 31% in TPP1E4/IVS5 NSCs to 47% in PPT1E8/E1 NSCs after 1 mM HPBCD treatment (b). The images were taken with 40X objective lens. Data are displayed as mean ± SD. * P < 0.05, ** P < 0.01, *** P < 0.001

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