Autophagy Promoted the Degradation of Mutant ATXN3 in Neurally Differentiated Spinocerebellar Ataxia-3 Human Induced Pluripotent Stem Cells

Figure 3

CAG repeats, genome, and mutant ATXN3 protein remained stable during reprogramming and neural differentiation. (a) Repeat sizes were confirmed by PCR with fluorescently labeled primers and capillary electrophoresis. The expanded alleles showed no detectable differences (15 (black arrows) and 81 (red arrows)) in fibroblasts (P3), SCA3 iPS cells (P15 and P30), NSCs (P3), and neurons (day 28) (yellow arrows: 30 repeats; green arrows: 23 repeats; blue arrows: 29 repeats). (b) Western blot bands from SCA3-iPS cells, NSCs, neurons, and control cells. P = passage. (c) No obvious de novo mutations in the fibroblasts (green), iPS cells (yellow), and the neurons (pink).

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