Figure 6.

Smcr8 deficiency exacerbates autophagy-lysosomal impairment in C9orf72^−/− spinal cords. (A, C, E) Representative confocal imaging of spinal cords from wild type (WT), C9orf72^−/−, Smcr8^−/− and C9orf72^−/−; Smcr8^−/− dKO mice at 7 months of age. Coronal sections were stained with antibodies against Lamp1 (red), NF (green), or p62 (green). Hoechst stains nuclei. White arrowheads represent axonal swellings with the co-localization of Lamp1 and NF. Scale bars: 20 μm. (B, D, F) Quantification of Lamp1-positive puncta per cell, numbers of NF aggregates per mm² area, or numbers of p62-positive puncta per cell (F). All data are presented as mean ± SEM using measurements averaged from ≥3 sections of each mutant mice (n = 3). Statistical analyses were performed with one-way ANOVA with Bonferroni correction’s post hoc test (^*P < 0.05, ^**P < 0.01); n.s. represents no significant difference detected.