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Figure 4 | Orphanet Journal of Rare Diseases

Figure 4

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

Figure 4

Morphometric analysis of differentiated cells obtained from hSKIN-MASC of healthy donors or NPC patients. (A- B): Phase contrast image showing the morphological differences between differentiated cells (5 days in N2 medium + 48 h in N3 medium, see methods) of a healthy donor (A) and a NPC patient (B). Specifically, NPC differentiated cells were characterized by distortion of neuron shape, ectopic neurites and meganeurite formation. (C): Quantitative analysis of neuronal morphological parameters linked to neurite outgrowth as evaluated by a specific Image analysis software on MAP-2 stained cells. Data are presented as mean ± SD of 3 independent experiments; §, p<0.01 vs healthy donors (CTRL).

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