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. 2020 Nov 2;16(11):e1009104. doi: 10.1371/journal.pgen.1009104

Fig 3. Reduction in striatal size and cerebellar abnormalities in R402H/+; Nestin-cre mice.

Fig 3

R402H/+ animals were crossed with a pan neuronal Nestin-cre driver line. (A-C) Representative coronal brain sections of +/+; Nestin-cre controls, R402H/+ controls, and R402H/+; Nestin-cre mutants at post-natal day 6 (P6) stained with Nissl. Dashed lines indicate the area measured for the ventricles (Ven) and striatum (Str). (D-E) Quantification revealed a significant difference in the striatal (D) and ventricular area (E) when comparing +/+; Nestin-cre controls, R402H/+ controls, and R402H/+; Nestin-cre mutants (n = 5, striatum: R402H/+ vs. R402H/+; Nestin-cre P<0.05, ventricle: +/+; Nestin-cre vs. R402H/+; Nestin-cre P<0.01). (F-H) Representative sagittal sections of the cerebellum at P6. (I) R402H/+; Nestin-cre mutants show a significant reduction of the cerebellar area compared to controls (n = 5, R402H/+ vs. R402H/+; Nestin-cre P<0.0001). (J-L) Representative cerebellar sagittal sections at P6 in which Purkinje cells were labeled with Calbindin (green) and neurons with NEUN (red). Sections were also counterstained with DAPI (in blue). (M-O) Enlargements of the boxed areas shown in (J-L). The internal granular layer (IGL), Purkinje cell layer (PCL), molecular layer (ML) and external granular layer (EGL) are marked. (P) Relative distribution of NEUN-positive neurons in the four layers of the cerebellum. There is a significant reduction in NEUN-positive cells found in the IGL and a significant increase in both the PCL and ML, in R402H/+; Nestin-cre mutants compared to littermate controls (n = 5, IGL: R402H/+ vs. R402H/+; Nestin-cre P<0.0001, PCL: R402H/+ vs. R402H/+; Nestin-cre P<0.01 and ML: R402H/+ vs. R402H/+; Nestin-cre P<0.0001). In all panels +/+; Nestin-cre controls are shown in light grey, R402H/+ controls in dark grey and R402H/+; Nestin-cre mutants in green. Error bars show the mean ± s.e.m. except for E, where data is represented as median ± 95% CI. *P<0.05, **P<0.01, ****P< 0.0001. Scale bars show 1000 μm in C, 500 μm in H, 200 μm in L and 40 μm in O.