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Fig. 1 | Acta Neuropathologica Communications

Fig. 1

From: Therapeutic targeting of Lyn kinase to treat chorea-acanthocytosis

Fig. 1

Vps13a−/− mice exhibit hematologic and neurologic features similar to those in human ChAc. a Left panel. Western blot (Wb) analysis of chorein in red cells of WT and Vps13a−/− mice. Catalase served as protein loading control. Lower panel. Densitometric analysis (arbitrary units) of immunoblot bands like those shown; means ± SEM (n = 6; *p < 0.001 vs. WT by t-test). Right panel (from left to right). Morphologic analysis of peripheral blood from wild-type (WT) and Vps13a−/− mice. Blood smears stained with May-Grunwald-Giemsa. Cells were imaged under oil at 100 × magnification using Panfluor objective 1.30 numeric aperture on a Nikon Eclipse DS-5 M camera and processed with Digital Slide (DS-L1) Nikon. Black arrows indicate acanthocytes in Vps13a−/− mice. (see also Additional file 1: Fig. S4.2C). Electron microscopy of circulating red cells from Vps13a−/− mice. The image is representative of 10 similar imaged visual fields for each of 10 Vps13a−/− mice at the ages of 12 months. b Western blot (Wb) analysis of Chorein in isolated basal ganglia of wild-type (WT) and Vps13a−/− mice. GADPH is the loading control. Densitometric analysis (arbitrary units) of the immunoblot bands similar to those shown are presented (bottom); data are means ± SEM (n = 6; *P < 0.001 vs. WT by t-test). c Spontaneous locomotor activity in Vps13a−/− and wild-type mice in undisturbed conditions. At 12 months mice were maintained in a PhenoTyper® cage (Noldus®) and continuously monitored for two consecutive days (Day 1 and Day 2). Data represent the mean ± SEM of the total distance moved (cm) per day (**P < 0.01 vs. wild-type mice, n = 6 animals per group). d CatWalk® gait analysis of Vps13a−/− and wild-type mice. The data represent the mean ± SEM of three runs per animal and are presented per each paw, left front (LF), left hind (LH), right front (RF) and right hind (RH) (*p < 0.05, **P < 0.01, ***P < 0.001; n: 19 Vps13a−/− mice, n: 15 wild-type mice) (see also Additional file 1: Fig. S5A). At 18 months of age Vps13a−/− mice showed a deviating paw angle of both hind paws compared to controls *P < 0.05; n = 19 Vps13a−/− mice, n = 15 wild-type mice)

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