Fig. 6

Fibrinogen deposition, presence of ataxin-3 aggregates in brain blood vessels and neuroinflammation in MJD patients. a Representative images of immunofluorescence with antibodies against CoIV in red and fibrinogen in green (Scale bars = 50 μm) suggestive of higher fibrinogen extravasation across BBB in striatum sections of 3 MJD patients when compared with 2 control individuals (summarized in graph b). c Quantification of CoIV surface area in striatum slices was similar between MJD patients and control individuals. d Representative co-immunofluorescence for ataxin-3 aggregates in green and CoIV in red, indicating the presence of ataxin-3 aggregates within CoIV-positive striatum blood vessels of MJD patients (Scale bar = 200 μm and 5 μm for orthogonal images). e Representative images of immunofluorescence to detect Iba1 in green, CoIV in red and GFAP in grey simultaneously, in striatum of both controls and MJD patients. Scale bars = 100 μm. f Iba1 is significantly increased in MJD patients (n = 3) comparing to controls (n = 2, Unpaired t test, P = 0.004). g No significant differences were detected regarding GFAP immnureactivity. Values of 2 controls and 3 MJD patients are presented as mean ± SEM. Unpaired t test with Welch’s correction, P > 0.05 = not significant; ** P < 0.01