Fig. 3: SEL1LS658P KI mice exhibit microcephaly and a mild reduction of Purkinje cells.
From: SEL1L-HRD1 interaction is required to form a functional HRD1 ERAD complex
a Representative pictures of brains of 5-week-old mice, with quantitation of brain weight shown on the right (n = 9, 4 and 9 mice for WT, HET and KI). b 3D rendering of cortex and cerebellum from 5-week-old mice, with quantitation shown on the right (n = 5 mice per group). c, d Hematoxylin & eosin (H&E) stained sagittal sections of whole brain (c) and cortex (d) from 5-week-old mice (n = 3 mice per group). Hippo., hippocampus; B. Stem, brain stem; Cereb., cerebellum. e Representative confocal images of NeuN (green), GFAP (red) and IbaI (purple) staining for neurons, astrocytes and microglia, respectively, in the cortex of 5-week-old mice (n = 2 mice per group).  f, g, h Hematoxylin & eosin (H&E) stained sagittal sections of the cerebellum from 5- (f) and 24- (g) week-old mice, with quantitation of Purkinje cell density shown in (h) (n = 5 mice for 5w WT and KI; n = 3 mice for 24w WT and KI). White arrowheads, Purkinje cells. ML, molecular layer; MW, white matter. i Western blot analysis of Calbindin in the cerebellum of 5- and 24-week-old mice with quantitation shown on the right (n = 6 mice for 5w WT and KI; n = 3 mice for 24w WT and KI). Values, mean ± SEM. n.s., not significant; *p < 0.05, ***p < 0.001 and ****p < 0.0001 by one-way ANOVA followed by Tukey’s post hoc test (a) and two-tailed Student’s t test (b, h, i).
