Skip to main content
Fig. 2 | Clinical Epigenetics

Fig. 2

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. 2

The bromodomain inhibitor PFI-1 enhances the expression of the C9ORF72 transcript variants. a Schematic representation of the BAC construct (153.2 kb) containing the human C9ORF72 gene promoter region (140.5 kb), exons 1 to 6 and a ~ 500 G4C2 hexanucleotide repeat expansions within intron 1 (red hexagons). The regions where each TaqMan probe hybridizes to detect the transcript variants V1 (blue), V2 (purple), V3 (orange) and Vall (green) are indicated. b–e TaqMan-based expression analyses of the V1 (b), V2 (c), V3 (d) and Vall (e) C9ORF72 transcripts. Samples were obtained from 10 DIV C9BAC primary cortical cultures treated with PFI-1 (5 µM in 0.05% DMSO) or vehicle DMSO (0.05%) for 24 h. Expression of each C9ORF72 transcript was normalized to DMSO treatments, and internally normalized to the expression of house-keeping gene Hprt. In all graphs, bars represent mean ± S.E.M. *P < 0.05, **P < 0.01 and ***P < 0.001 relative DMSO treated cells, unpaired Student’s t-test (n = 3 independent experiments)

Back to article page