Fig. 2.

Characterization and differentiation of patient-derived NLSDM-iPSC.

(A) Immunostaining with primary antibodies anti-OCT4, anti-SSEA-4 and anti-TRA-1-81 revealed that NLSDM-iPSCs express markers of pluripotency, including OCT4 (red), SSEA4 (green), and TRA-1-81 (green). Nuclei were stained with DAPI. Magnification: 10 ×.

(B) Real-time quantitative RT-PCR assays for eight pluripotency-associate genes in two patient-derived iPSCs compared with fibroblast cell lines (basal level reported as 1). Data represent the mean of three independent experiments, performed in triplicate. PCR reactions were normalized against an internal control (ACTB).

(C) Quantification of TG content in iPSCs derived from 2 controls and 2 NLSDM fibroblast cell lines. The graph represents TG content obtained from three independent experiments. The analysis was performed using Student t-test. Thick bar: mean value; error bar: SD. P-values of < 0.05 and of < 0.01 were considered to be significant and indicated with “*”and “**”, respectively.