Fig. 1: Ventriculomegaly in Kidins220 hypomorphic mice.
From: Kidins220 deficiency causes ventriculomegaly via SNX27-retromer-dependent AQP4 degradation
A Total number (n = 232) and percentage of WT, Kidins220f/+ and Kidins220f/f mice alive at weaning vs their expected Mendelian ratio. B Percentage of WT, Kidins220f/+ and Kidins220f/f mice death at weaning showing severe hydrocephalic phenotype (n = 232). C Representative images of 2-month-old WT, Kidins220f/f, and severely hydrocephalic Kidins220f/f (Kidins220f/f(S)) mice. Right: Zoom detail of the head. D Representative in vivo T2-weighted (T2-W) MRI coronal images showing lateral and third ventricles (LVs + TV; left panels), aqueduct of Sylvius (SA; central panels—dotted line) and fourth ventricle (FV; right panels—dotted line) of 2-month-old WT, Kidins220f/+, Kidins220f/f and Kidins220f/f(S) male mice. Scale bar, 1 mm. Quantification of ventricular and aqueduct volume (mm3) in 17 WT (circles), 6 Kidins220f/+(squares) and 14 Kidins220f/f (triangles) mice. LVs and TV values from the same group of 17 WT (circles) animals have been represented in a separate graph with those of three severe Kidins220f/f(S) (diamonds) mice. E Apparent Diffusion Coefficient (ADC) representative coronal images showing LVs and TV (left panels), SA (central panels—dotted line) and FV (right panels—dotted line) of 2-month-old WT, Kidins220f/f and Kidins220f/f(S) male animals. The pseudo-colour scale indicates maximum and minimum ADC values in μm2/s. Scale bar, 1 mm. Quantification of ADC values (μm2/s) at the ventricles and aqueduct in WT (circles; LVs, TV and SA, n = 16; FV, n = 12), Kidins220f/f (triangles; LVs, TV and SA, n = 12; FV, n = 8) and Kidins220f/f(S) (n = 3, diamonds) mice. F Representative Kidins220 and α-tubulin (loading control) immunoblots of cortex (Cx) and cerebellum (Cb) lysates from WT, Kidins220f/+ and Kidins220f/f mice; each lane represents one animal. Kidins220 levels are represented in arbitrary units after normalization with α-tubulin in Kidins220f/+ (Cx, n = 6; Cb, n = 4) and Kidins220f/f (Cx, n = 6; Cb, n = 5) relative to WT (Cx, n = 7, Cb, n = 4) mice. G Correlation between Kidins220 relative levels in the cortex (Cx, left panel) and cerebellum (Cb, right panel) represented in (F) and LVs and TV volumes quantified in T2-W MRI images depicted in (D), established by linear regression analysis (Cx, R2: 0.5954; Cb, R2: 0.6231). D, E, F Data are mean ± s.e.m.; each data point denotes an individual mouse; NS not significant; ***P < 0.001, by one-way ANOVA (D, F) and two-tailed unpaired Student’s t test (E).
