Fig. 2.

Neural differentiation of FHB1 and FH10 ( Y218N ) iPS cells. IPS cells from control (FHB1) and Y218N (FH10) GSS patient were differentiated using two procedures (a and e) (see Methods for details). (b) Low power photomicrographs of representative colonies of FHB1 and FH10 (Y218N) stained positive for SOX2, Nestin, DCX, TUJ1, PAX6, UBE3A antigens at different stages of maturation. (c) Western blot characterization of PrP^C expression in differentiating iPS cell cultures. (d) Example of the Western blot experiments illustrating the absence of PK-resistant PrP^C in FH10 (Y218N) cultures. (e) Low power photomicrographs of representative colonies of FHB1 and FH10 (Y218N) stained positive for Nestin, Ki67, SOX2, TUJ1, PAX6 and GFAP antigens. (g) Western blot characterization of PrP^C expression in iPS cells (passage 20) and SNMs (passage 3). (h) Western blots illustrating the absence of PK-resistant prion in FHB1 and FH10 (Y218N) in brain extracts from the GSS patient and two CJD (Type I and II) samples. Scale bars in b and f = 50 μm