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. 2024 Apr 22;14:9177. doi: 10.1038/s41598-024-59834-6

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© The Author(s) 2024

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Investigation of GD phenotypes in iPSCs-derived neuronal cells acquired from neuronopathic GD. (a) (Left) Representative western blot images of GBA1 and beta actin from iPSCs-derived neuronal cells from control, GD3-1 and GD3-2 samples. (Right) Bar graph summarizing of the relative normalized GBA1 protein expression in GD3-1 and GD3-2 compared to control group (Kruskal–Wallis test: H(2) = 10.14, p = 0.04 for Control vs. GD3-1, p < 0.01 for Control vs. GD3-2, n = 5 technical replicates). (b) Bar graphs representing the relative glucocerebrosidase activity in iPSCs-derived neuronal cells (right, Kruskal–Wallis test: H(2) = 8.01, p = 0.04 for Control vs. GD3-1, p < 0.01 for Control vs. GD3-2, n = 4 technical replicates) in GD3-1 and GD3-2 compared to control group. (c) Representative cropped western blot images of LAMP1, LC3I/II and GAPDH from iPSCs-derived neuronal cells from control, GD3-1 and GD3-2 samples (all replicate images are included in the supplementary information file). (d) Bar graph summarizing of the relative normalized LAMP1(left, Kruskal–Wallis test: H(2) = 5.79, p = 0.02 for Control vs. GD3-1, p = 0.07 for Control vs. GD3-2, n = 3 technical replicates) and LC3I/II (right, Kruskal–Wallis test: H(2) = 6.16, p = 0.02 for Control vs. GD3-1, p = 0.10 for Control vs. GD3-2, n = 3 technical replicates) protein expression in GD3-1 and GD3-2 compared to control group. (e) (Top) Representative images of SiR lysosome staining in iPSCs-derived neuronal cells from control, GD3-1 and GD3-2 samples. (Bottom left) Expanded micrograph of boxed region demarcated in the corresponding images. (f) Summary of the relative average SiR lysosome staining per cell in GD3-1 and GD3-2 compared to control group (Kruskal–Wallis test: H(2) = 14.9, p = 0.04 for Control vs. GD3-1, p < 0.01 for Control vs. GD3-2, n = 6 technical replicates). (g) (Top) Representative images of acridine orange staining in iPSCs-derived neuronal cells from control, GD3-1 and GD3-2 samples. (Bottom) Expanded micrograph of boxed region demarcated in corresponding images. (h) Summary of the relative average acridine orange staining per cell in GD3-1 and GD3-2 compared to control group (Kruskal–Wallis test: H(2) = 7.69, p = 0.04 for Control vs. GD3-1, p = 0.03 for Control vs. GD3-2, n = 4 technical replicates).