Fig. 3: Cl-amidine reduced perivascular hematomas and anti-coagulant proteins in CCM.

a, Representative images of a normal clot (left) and a perivascular hematoma (right) in the cerebellum of Ccm3-iECKO vehicle-treated mice stained with DAPI (blue), TER-119 (green) and podocalyxin (white). b, Quantification of percentage of mice with perivascular hematomas in Ccm3-iECKO vehicle-treated (n = 13) and Cl-amidine-treated (10 mg kg−1 d−1; n = 8) mice. Statistical significance was analyzed with Fisher’s exact t-test. c,d, Quantification of the number and area of perivascular hematomas in the cerebellum of Ccm3-iECKO vehicle-treated (n = 13) and Cl-amidine-treated (10 mg kg−1 d−1; n = 8) mice. e, 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 THBD (red) and ILB4 (cyan). Insets with dashed, white line are shown in the magnifications to the right. 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 annexin A5 (green) and ILB4 (white). Insets with dashed, white line are shown in the magnifications to the right. g,h, Quantification of vascular (ILB4+) THBD expression (g) and vascular (ILB4+) annexin A5 expression (h) 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 c,d and in g,h, 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; P. hematoma, perivascular hematoma; THBD, thrombomodulin.