Fig. 2: iPSC-derived neurons from C9-HRE-carrying FTD patients express typical C9-HRE-associated pathological hallmarks. | Molecular Psychiatry

Fig. 2: iPSC-derived neurons from C9-HRE-carrying FTD patients express typical C9-HRE-associated pathological hallmarks.

From: Frontotemporal dementia patient-derived iPSC neurons show cell pathological hallmarks and evidence for synaptic dysfunction and DNA damage

Fig. 2

Fluorescence in situ hybridization (FISH) shows that C9-HRE neurons but not sporadic FTD or control neurons exhibit sense RNA foci (red) as detected using the TYETM 563-(CCCCGG)3 probe (A). Image analysis reveals that approximately 17% of neurons display sense RNA foci (B). The neurons contain on average two RNA foci (C). Representative microscopy images are shown. Also, C9-HRE carrying neurons specifically produce poly-GP (D) and poly-GA (E) DPR proteins. One-way ANOVA followed by Tukey’s multiple comparisons test was used. n [Control] = 11; n [C9−] = 8; n [C9+] = 5. C9-HRE-carrying neurons do not present with C9orf72 protein haploinsufficiency but a slight non-significant trend towards lower C9orf72 protein levels as compared to controls (F). One-way ANOVA followed by Tukey’s multiple comparisons test was used. n [Control] = 35; n [C9−] = 61; n [C9+] = 46. Statistically significant differences are shown as ***p < 0.001 and ****p < 0.0001.

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