The blood-brain barrier is disrupted in Machado-Joseph disease/spinocerebellar ataxia type 3: evidence from transgenic mice and human

摘要

EB extravasation reveals BBB leakage in MJD mice. a Schematic presentation of the experimental set for EB injection (n = 4 MJD transgenic, Tg and n = 6 wild-type, WT animals; 11 month-old). b EB concentration (pg/μL) in the cerebrum showed no differences between wild-type and transgenic animals. c Cerebellum, on the other hand, exhibited a 5-fold increase of EB in MJD transgenic mice (Unpaired t test; P = 0.02). d, e Representative fluorescence images of the cerebellum of wild-type and transgenic mice revealed EB deposition mainly in deep cerebellar nuclei (DCN) and lobule 8 and 9, respectively, of transgenic animals (Scale bars = 50 μm). Values are presented as mean ± SEM. Unpaired t test with Welch’s correction, P > 0.05 = not significant and *P ≤ 0.05