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. 2010 May 7;6(5):407-11.
doi: 10.1016/j.stem.2010.04.005.

Differential modeling of fragile X syndrome by human embryonic stem cells and induced pluripotent stem cells

Achia Urbach  1 , Ori Bar-Nur, George Q Daley, Nissim Benvenisty
Affiliations

Differential modeling of fragile X syndrome by human embryonic stem cells and induced pluripotent stem cells

Achia Urbach et al. Cell Stem Cell. .
No abstract available

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Figures

Figure 1
Figure 1. FMR1 expression in FX-iPS cells
A. FMR1 transcript expression as analyzed by real-time PCR in FX-ES cells, normal ES cells, normal fibroblasts, two normal iPS cell clones, FX-fibroblasts from three different patients (A, B, C), and eleven FX-iPS cell clones derived from the FX-fibroblasts. B. Comparison of FMR1 expression levels in two FX-iPS cell clones at low passage (p5) and high passage (p17 and p18). C. Immunostaining for FMRP in ES, iPS, FX-ES and FX-iPS cells using goat anti human FMRP antibody and Hoechst 33258 for nuclear staining. D. FMR1 and NANOG expression levels in FX-ES cells, Diff-FX-ES cells (a population of cells differentiated from FX-ES cells), and Diff-FX-ES-iPS cells (iPS cells derived from differentiated FX-ES cells). E. Analysis of CGG repeat number in Diff-FX-ES, Diff-FX-ES-iPS clones #2 and #6, two samples of FX-ES cells, and two subclones of the FX-ES cells. Note that the Diff.-FX-ES and the FX-ES represent cell populations that are heterogeneous with respect to CGG repeat number (Eiges et al, 2007), whereas in the Diff-FX-ES-iPS and the FX-ES subclones a dominant band indicates the more homogenous population expected in subclones. The size of the DNA marker bands in base pairs and the number of the CGG repeats are shown at the left and right sides of the gel, respectively. For further characterization of the undifferentiated iPS clones and their pluripotency see also supplementary figures 1,2.
Figure 2
Figure 2. Epigenetic modifications in FX-iPS cells
A. Pyrosequencing analysis of the FMR1 promoter in wt-fibroblasts, two clones of wt-iPS cells (iPS 28, iPS 94), FX-fibroblasts-A, and seven derivative iPS cell lines (FX-iPS A-12, 17, 47, 50, 52, 55, 89), FX-fibroblasts-C and two derivative iPS cell lines (FX-iPS C-2, C-3). Upper panel – methylation level at CpG position. Bottom panel – average methylation level of all CpG sites for each sample. B. Bisulfite sequencing analysis of the OCT4 promoter in FX-fibroblasts-A and FX-iPS cell clones derived from them. Open circles, unmethylated CpGs; Black circles, methylated CpGs. C. Pyrosequencing analysis of the OCT4 5’UTR in FX-fibroblasts-C and FX-iPS cell clones derived from them. D. Histone modifications at the FMR1 locus in FX-iPS cells: ChIP analysis of histone H3-tail acetylation and H3K4 and H3K9 methylation in FX-iPS cells. Real-time PCR was performed on bound and input sonicated DNA fragments using primers for the FMR1 promoter. Adenine phosphoribosyl transferase (APRT) and Crystalline (CRYST) served as positive and negative controls, respectively. Values were normalized to the appropriate positive control and shown with their respective standard errors.
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