(A) Images of GM03816 FRDA fibroblasts (left), GM03816 iPSCs (middle), and H1 hESCs (right). Scale bars = 0.5 mm.
(B) GM03816-iPS4 shows similar expression of pluripotency mRNAs as H1 hESCs. GM03816 fibroblasts, white bars; GM03816 iPSCs, light grey; H1 hESCs, dark grey. mRNA levels are normalized to GAPDH. Error bars = SEM of duplicate measurements.
(C) Hallmark repression of FXN mRNA in GM03816 fibroblasts and the GM03816-iPS4 line as compared to the unaffected H1 line. Error bars = SEM of duplicate measurements.
(D) GM03816-iPS4 and H1 hES staining (contrast enhanced) of pluripotency markers. Phase contrast (gray); nuclear staining (blue); pluripotency markers staining (green and red) is as denoted by the colored text labels. Tra1–60 and Tra1–81, surface markers; SSEA-3 and -4, stage-specific embryonic antigens; Oct4, transcription factor. Scale bars = 0.25 mm.
(E) Microarray analysis of FRDA iPSCs versus unaffected iPSC/ESCs, human tissues, and cell lines. Red represents up-regulation, green represents down-regulation. Unaffected iPS/ESCs, red highlighting; FRDA iPSCs, yellow; unaffected human tissue, blue; human cell lines, gray. See also .