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Fig. 5 | Acta Neuropathologica Communications

Fig. 5

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

Fig. 5

Expression level of TJ-associated proteins in the cerebellum of MJD mice differ from wild-type controls. a Schematic representation of TJ structure in BBB (juncle adhesion molecules, JAMs). b Representative western blot membrane of TJ-associated proteins in the cerebellar protein extracts of transgenic and wild-type (WT) mice at 16–17.5 months old. c-h Western blot quantification of TJ-associated proteins. c Relative levels of occludin (63 kDa) were similar in the two groups, but the 55 kDa occludin fragment including the C-terminal (d) was strongly present (Unpaired t test, P = 0.0025) in the cerebellum of transgenic mice (n = 6), when compared to wild-type mice (n = 7). e Levels of expression of a band of 100 kDa, corresponding to claudin-5 oligomers, exhibited a 29% decrease (Unpaired t test, P = 0.05) in MJD mice. On the contrary, claudin-5, as a monomer of 27 kDa (f), showed a 2-fold increase (Unpaired t test, P = 0.005) in transgenic (n = 7) relatively to wild-type mice (n = 7). g Relative levels of ZO-1 α- isoform showed no significant differences between transgenic (n = 4) and 4 wild-type (n = 4) mice. h CD31 levels are similar in both wild-type and transgenic animals indicating equal amounts of endothelial cells and blood vessels in the cerebellum of both groups. All protein relative levels were normalized with GAPDH. Values are presented as mean ± SEM. Unpaired t test with Welch’s correction, P > 0.05 = not significant; * P ≤ 0.05; ** P < 0.01

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