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Fig. 3 | Clinical Epigenetics

Fig. 3

From: BET bromodomain inhibitors PFI-1 and JQ1 are identified in an epigenetic compound screen to enhance C9ORF72 gene expression and shown to ameliorate C9ORF72-associated pathological and behavioral abnormalities in a C9ALS/FTD model

Fig. 3

PFI-1 treatments reduce poly(GP) levels in C9BAC primary cortical neurons. a Representative confocal images of fixed 10 DIV cortical neurons derived from C9BAC (C9BAC) and non-transgenic (Ntg) mice analyzed by immunofluorescence labeling with an antibody against MAP2 (red) to identify neurons and an antibody against poly(GP) (green) to identify poly(GP) DPRs. DAPI (blue) staining identified the nuclei. Images show abundant presence of poly(GP) DPRs in C9BAC, but not in Ntg, cortical neurons. Poly(GP) DPRs are strongly reduced after treatment with PFI-1 (5 µM in 0.05% DMSO). Pre-incubation of the anti-poly(GP) antibody with a poly(GP) peptide led to a significant loss of the Poly(GP)-DPR signal. b Quantification of the nuclear fluorescence intensity at each indicated condition, normalized to the intensity in Ntg cells. Bars represent mean ± S.E.M. ***P < 0.001 relative to untreated C9BAC cells, one-way ANOVA. (n = 3 independent experiments with a minimum of 30 nuclei quantified for each condition)

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