Fig. 1
From: Expression of expanded GGC repeats within NOTCH2NLC causes cardiac dysfunction in mouse models
Expression of NOTCH2NLC-(GGC)98 produces polyG inclusions in the cardiomyocytes of EIIa-tg mice. A Schematic diagram of NOTCH2NLC-(GGC)98 construct. The conditional transgenic mice were obtained by inserting the loxP-stop-loxP-NOTCH2NLC-(GGC)98 into the Rosa26 site. B Immunoblotting with an anti-Flag antibody in the hearts of control and EIIa-tg mice at P30. Vinculin was used as a loading control. C Immunofluorescent staining against Flag in the hearts of control and EIIa-tg mice at P30. Red, flag (polyG); cyan, DAPI. Scale bar, 50 μm. D Co-immunofluorescent staining against Flag and p62 in the hearts of control and EIIa-tg mice at P30. Red, p62; Green, flag (polyG); cyan, DAPI. Scale bar, 10 μm. E Colocalization of p62 with polyG inclusions in the heart of EIIa-tg mice. N = 3 mice per group, three images per mouse were used to count