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Figure 2 | Orphanet Journal of Rare Diseases

Figure 2

From: A human neuronal model of Niemann Pick C disease developed from stem cells isolated from patient’s skin

Figure 2

Neural differentiation of hSKIN-MASC obtained from already established skin fibroblast cultures. (A-B) Phase contrast images of hSKIN-MASC after induced to differentiate toward a neuronal fate (5 days in N2 medium + 48 h in N3 medium, see methods) from a healthy donor (A) and a NPC patient (B). (C-H) Neuronal markers detection: differentiated cells (5 days in N2 medium + 48 h in N3 medium, see methods), obtained from a healthy donor (C, F) or a NPC patient (D, G), express the neuron specific markers NeuN (yellow fluorescence, C, D) and MAP2 (green fluorescence, F, G). Nuclei are depicted by the blue fluorescence of DAPI staining. Quantitative evaluation of the percentage of cells expressing NeuN (E) or MAP2 (H) in cultures from healthy donors (CTRL, n = 3) and NPC patients (n = 3) skin fibroblast derived cultures, before (Undiff.) and after the exposure to neuron induction media (Diff.). At least 400 cells have been counted for each cell line. Data are presented as mean ± SD of 3 independent experiments one-way Anova test followed by Bonferroni post-test were utilized to compare means between groups. P values less than 0.05 were considered significant. P values less than 0.05 were considered significant. *, **, ***, p<0.05 vs. columns 1, 2, and 3, respectively.

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