Fig. 3: CA16LD and XE1AH fibroblasts show a defect in GGR function. | European Journal of Human Genetics

Fig. 3: CA16LD and XE1AH fibroblasts show a defect in GGR function.

From: A new multisystem ERCC1-hepatorenal syndrome: insights from a clinical cohort, molecular pathogenesis, and management guidelines

Fig. 3: CA16LD and XE1AH fibroblasts show a defect in GGR function.

A Representative microscopic images of nuclei (DAPI), local UV damage (CPD), and unscheduled DNA synthesis (EdU), and B quantification of the EdU intensity at the site of local damage in ERCC1 patient fibroblasts after local UV-C irradiation. EdU is incorporated for 1 h after 30 J/m² UV irradiation. The intensity of EdU is normalized to wildtype 48BR. Each datapoint represents one local damage site, with the colored bar depicting the mean of all datapoints, and the colored points representing the mean of the individual replicates. The scale bar is 10 µm. Statistical significance was determined by a paired 2-tailed t-test (* = p < 0.05).

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