Additional file 1 of Reversibility of motor dysfunction in the rat model of NGLY1 deficiency

Additional file 1: Table. Raw data of rota-rod tests in each animal at each age. Figure 1. AsnGlcNAc levels in the brain of 11-week-old viral-injected WT or Ngly1−/− rats. Values represent means. One rat is examined in each group.  Figure 2. Plasma AST (a) and ALT (b) levels of 11-week-old virally injected WT or Ngly1−/− rats. Values represent means ± S.E.M. The number of rats examined is shown in parentheses. n.s. means no significance. Figure 3. Stride lengths of 5-week old rats in gait analysis 2 weeks after AAV-injection. Values represent means ± S.E.M. The number of rats examined is shown in parentheses. n.s. means no significance. Figure 4. Grip-strength tests for assessment of 2paws (a) or 4paws (b) muscle force of 11-week-old AAV-injected rats. Values represent means ± S.E.M. The number of rats examined is shown in parentheses. n.s. means no significance. Figure 5. Body weight of WT and Ngly1−/− rats. Rats were weighed weekly after AAV administration. The number of rats examined is shown in parentheses. n.s. means no significance. Figure 6. Neuronal degeneration in the thalamus of Ngly1−/− rats was not recovered in AAV9-hNGLY1 injected Ngly1−/− rats. H&E-stained sections of the thalamus from the WT and Ngly1/rats 8 weeks after AAV administration. Scale bar 250 μm. Figure 7. (a) Immunohistochemistry for ubiquitin in the thalamus of virus-injected WT and Ngly1−/− rats at 11 weeks of age. Scale bar, 250 μm. Nuclei were stained with DAPI. (b) Accumulation of polyubiquitinated proteins in spinal cords of Ngly1−/− and WT rats. The total protein extracts were separated from the spinal cords of the rats and analyzed by immunoblotting using anti-polyubiquitinated antibodies (top) and anti-GAPDH antibodies (bottom; loading control). (c) Semi-quantitative analyses by densitometry were carried out. Values represent mean ± SEM (Ngly1−/− rats + empty; n = 5, Ngly1−/− rats + hNGLY1; n = 3). n.s. = no significance.