Fig. 4: Cl-amidine reduced platelet activation and coagulation in CCM.

a, Representative images of the cerebellum of Ccm3-iECKO vehicle-treated mice (upper panel) and Cl-amidine-treated (10 mg kg−1 d−1) mice (lower panel) stained with DAPI (blue), vWF (green) and podocalyxin (white). Insets with dashed, white line are shown in the magnifications to the right. b, Quantification of vWF+ area in the cerebellum of Ccm3-iECKO vehicle-treated (n = 13) and Cl-amidine-treated (10 mg kg−1 d−1; n = 8) mice. c, Representative images of the cerebellum of Ccm3-iECKO vehicle-treated mice (upper panel) and Cl-amidine-treated (10 mg kg−1 d−1) mice (lower panel) stained with DAPI (blue), CD41(magenta) and CD42b (green). Insets with dashed, white line are shown in the magnifications to the right (lesion periphery is shown with blue lines). d, Quantification of CD41+ area in the cerebellum of Ccm3-iECKO vehicle-treated (n = 13) and Cl-amidine-treated (10 mg kg−1 d−1; n = 8) mice. e, Quantification of total platelet (CD41+) area that is activated (CD42b+) in the cerebellum of Ccm3-iECKO vehicle-treated (n = 12) and Cl-amidine-treated (10 mg kg−1 d−1; n = 8) mice. f, Representative images of the cerebellum of Ccm3-iECKO vehicle-treated mice (upper panel) and Cl-amidine-treated (10 mg kg−1 d−1) mice (lower panel) stained with DAPI (blue), TER-119 (erythrocytes, green), fibrin (red) and ILB4 (white). g–i, Quantification of fibrin+ area (h), clot area (defined as lumens with fibrin and erythrocytes; g) and TER-119+ area (i), in the cerebellum of Ccm3-iECKO vehicle-treated (n = 13) and Cl-amidine-treated (10 mg kg−1 d−1; n = 8) mice. In the graphs, each data point represents one biological replicate; the bar indicates the median of each group; and the error bars represent the IQR. Statistical significance was determined using a Mann–Whitney U-test (two-tailed). cb, cerebellum.