Figure 4.

MeCP2^G273X-EGFP Mice Appear Unaffected by a Mutation Inactivating the MeCP2 NLS

(A) Survival curves for Mecp2^G273X-EGFP (n = 8) and Mecp2^G273XΔNLS-EGFP mice (n = 9), as well as wild-type controls for each group (n = 12 and n = 10, respectively). No significant difference between survival of Mecp2^G273X-EGFP and Mecp2^G273XΔNLS-EGFP mice was observed (p = 0.78; Kolmogorov-Smirnov test). An illustrative survival curve for Mecp2-null mice (based on data from previous studies) is shown as a broken line.

(B) Average phenotypic severity score of surviving animals plotted against age for each of the groups. Data for each group are plotted up to the last point, where there are at least three surviving animals.

(C) Expression levels relative to Gapdh of genes that are upregulated (Flrt3 and Arhgap10) or downregulated (Sst, Tspan17, and Gabbr1) in brains of Mecp2 mutant mice as assessed by qPCR. Data are presented as means ± SD.

(D) Brain weights for Mecp2^G273X-EGFP and Mecp2^G273XΔNLS-EGFP mice (n = 4 for each group) as well as wild-type controls (n = 5 and n = 3). Indicated statistical significance was calculated using the t test. Data are presented as means ± SD.

(E) Cortical thickness plotted for Mecp2^G273X-EGFP and Mecp2^G273XΔNLS-EGFP mice (n = 4 each) and wild-type control animals (n = 5 and n = 3). Data are presented as means ± SD. Indicated statistical significance was calculated using the t test. The image depicts the area of the primary motor cortex (M1) measured. The scale bar represents 1,000 μm.

See also Figure S2.