Sting1 ^−/− ameliorates neurological disease of iNgly1 ^−/− mice. (A) Schematic diagram showing generation of iNgly1^−/−Sting1^−/− mice. (B) Survival curves of Ngly1^fl/fl, Ngly1^fl/flSting1^−/−, iNgly1^−/−, and iNgly1^−/−Sting1^−/− male mice. Log-rank (Mantel–Cox) test. **P < 0.01; ***P < 0.001. (C) Neurological deficit score of Ngly1^fl/fl, Ngly1^fl/flSting1^−/−, iNgly1^−/−, and iNgly1^−/−Sting1^−/− male mice. Data were shown as median ± 95% CI. Mann–Whitney test. (D) IHC staining of calbindin in cerebella of 1-year-old Ngly1^fl/fl, Ngly1^fl/flSting1^−/−, iNgly1^−/−, and iNgly1^−/−Sting1^−/− mice. Scale bar, 500 μm (upper), 100 μm (lower). (E) Quantification of Purkinje cell number of indicated genotypes. Data were shown as the mean ± SEM. One-way ANOVA with Bonferroni’s multiple comparisons test. **P < 0.01; ***P < 0.001. (F and G) IHC staining of cleaved caspase-3 (cl-Casp3, green) and calbindin (red) in cerebella of 8-wk-old Ngly1^fl/fl, Ngly1^fl/flSting1^−/−, iNgly1^−/−, and iNgly1^−/−Sting1^−/− mice. Scale bar, 50 μm. Quantification of cl-Casp3⁺ Purkinje cells per midsagittal cerebellar section is shown in G. Data are from mice (n = 12 per genotype) of three independent experiments. Data were shown as the mean ± SEM. One-way ANOVA with Bonferroni’s multiple comparisons test. ***P < 0.001.