Recapitulation of Disease Phenotypes in the Diseased iENPs and Their Neuronal Derivatives
(A) Representative images of the morphology and ICC staining for Nestin in (a) AD-iENPs and (b) HD-iENPs.
(B) Phase-contrast image of AD-iENP-derived (a) and HD-iENP-derived (b) neurons and ICC staining of AD-iENP (a) and HD-iENP (b) derivatives using antibodies against GFAP, GALC, and TUJ1.
(C) Secreted Aβ42/40 ratio; Aβ42 and Aβ40 from AD-iENP-derived neurons. AD2 and AD3, patients carrying PSEN1 mutations.
(D) (a) ICC staining analysis of pTAU expression in AD-iENP-derived neurons using antibodies against TUJ1 and pTAU (AT8). (b) Quantification of the effect of 1-Aza and SB415286 on the reduction in pTAU expression in AD-iENP-derived neurons. AD1, patient carrying the APOE4/E4 mutation. Controls were treated with DMSO. Arrowheads indicated pTAU accumulation.
(E) ICC staining (a) and quantification (b) of γH2AX+ cells in vehicle (DMSO)- and CGS 21680-treated control and HD-iENPs. (c) ICC staining and quantification of γH2AX+ cells in vehicle (DMSO)- and CGS 21680-treated controls and HD-iENP-derived neurons. Arrows indicated γH2AX+ cells.
All quantitative data were obtained from three independent experiments and are expressed as means ± SD. ∗p < 0.05. See also Figure S5.