Mitochondrial and Peroxisomal Alterations Contribute to Energy Dysmetabolism in Riboflavin Transporter Deficiency

Figure 2

Morphological features of Ctrl and RTD iPSCs. (a) Phase contrast images show absence of typical colonies in diseased cells as compared to controls. Scale bars, 20 μm. (b) Confocal analysis after immunofluorescence using ZO-1 marker (green) demonstrates abnormal cell-cell contacts in RTD (ii, iii, v, and vi) as compared to Ctrl (i, iv) iPSC. White arrows indicate ZO-1 aggregates in RTD iPSCs P1 (vi, vii) while white arrowheads indicate immunofluorescence puncta (v, vi). Scale bars, 10 μm (i–iii), 5 μm (iv–vi). Nuclei are stained with Hoechst (blue). (c) Fluorescence intensity levels reveal a significant decrease of ZO-1 expression in RTD vs. Ctrl iPSCs ).