Fig. 3.

Disease phenotype of mouse iPS cells. (A) Defects of specific enzyme activities in disease model–derived iPS cells were confirmed by enzyme assay. The numbers in the disease model–derived iPS are individual clone numbers. Data for iPS cells are presented as mean ± SD (n = 3). (B) Accumulated Gb3 in Fabry-iPS cells was confirmed by immunostaining. (Left) A colony of undifferentiated Fabry-iPS cells grown on the feeder cells is positive for Gb3. (Right) Confocal laser scanning microscopy confirmed positive signals in cytoplasm. [Scale bars: 200 μm (Left); 20 μm (Right).] (C) Analysis of Gb3 by TLC. (D) Elevated HA levels in EBs derived from MPSVII-iPS cells (two individual clones) was confirmed by HPLC.