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Fig. 1 | Molecular Brain

Fig. 1

From: RT2 PCR array screening reveals distinct perturbations in DNA damage response signaling in FUS-associated motor neuron disease

Fig. 1

DNA repair and DDR gene expression profile by RT2 profiler PCR array in FUS knock out (KO) and knockdown (KD) cells, and its verification in ALS patient-derived motor neurons with a FUS P525L mutation reveal a complex perturbation pattern. a Heat map showing altered expressions of DNA repair genes in FUS KO cells. Red, green, and black squares indicate up-regulated genes, down-regulated genes, and non-regulated genes, respectively. b Scatter plot showing genes with > 2-fold difference in mRNA expression in FUS KO cells compared to control. Red, green, and black circles indicate up-regulated genes, down-regulated genes, and non-regulated genes, respectively. c Bar graph showing repair genes that were commonly down-regulated > 2 fold in FUS KO and KD cells compared to the control. d Histogram showing the relative mRNA expression level in FUS WT, FUS KO and FUS KD HEK293 cells. e Immunofluorescent labeling of motor neurons differentiated from ALS patient-derived iPSC lines for the indicated marker proteins. Representative images labeled for FUS shown cytoplasmic accumulation of FUS P525L mutant motor neurons. Labeled for Isl-1 and MAP 2 indicated ~ 80% differentiation efficiency of FUS WT and FUS P525L mutant iPSCs. Nuclei are stained with DAPI. f IB of endogenous FUS, BRCA1, MSH2, LIG4, and RAD23B in FUS WT and FUS P525L motor neurons. Histogram shows band intensity quantitation. *, p < 0.01. Error bars represent standard deviation from three independent experiments

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