Biogen, 225 Binney St., Cambridge, MA, 02142, USA.
Astellas, 1030 Massachusetts Avenue, Cambridge, MA, 02138, USA.
Sci Rep. 2019 Jul 1;9(1):9477. doi: 10.1038/s41598-019-45676-0.
Despite Bridging INtegrator 1 (BIN1) being the second most statistically-significant locus associated to Late Onset Alzheimer's Disease, its role in disease pathogenesis remains to be clarified. As reports suggest a link between BIN1, Tau and extracellular vesicles, we investigated whether BIN1 could affect Tau spreading via exosomes secretion. We observed that BIN1-associated Tau-containing extracellular vesicles purified from cerebrospinal fluid of AD-affected individuals are seeding-competent. We showed that BIN1 over-expression promotes the release of Tau via extracellular vesicles in vitro as well as exacerbation of Tau pathology in vivo in PS19 mice. Genetic deletion of Bin1 from microglia resulted in reduction of Tau secretion via extracellular vesicles in vitro, and in decrease of Tau spreading in vivo in male, but not female, mice, in the context of PS19 background. Interestingly, ablation of Bin1 in microglia of male mice resulted in significant reduction in the expression of heat-shock proteins, previously implicated in Tau proteostasis. These observations suggest that BIN1 could contribute to the progression of AD-related Tau pathology by altering Tau clearance and promoting release of Tau-enriched extracellular vesicles by microglia.
尽管桥接整合因子 1(BIN1)是与晚发性阿尔茨海默病相关性第二显著的基因座,但它在疾病发病机制中的作用仍需阐明。由于有报道称 BIN1 与 Tau 和细胞外囊泡之间存在关联,我们研究了 BIN1 是否可以通过外泌体分泌影响 Tau 的传播。我们观察到,从受 AD 影响个体的脑脊液中纯化的 BIN1 相关 Tau 包含的细胞外囊泡具有成核能力。我们表明,BIN1 过表达可促进 Tau 通过细胞外囊泡在体外释放,并在 PS19 小鼠体内加剧 Tau 病理学。在 PS19 背景下,从小胶质细胞中遗传删除 Bin1 可导致体外通过细胞外囊泡分泌 Tau 减少,以及雄性而非雌性小鼠体内 Tau 传播减少。有趣的是,在雄性小鼠的小胶质细胞中删除 Bin1 会导致热休克蛋白的表达显著减少,这些蛋白先前与 Tau 稳定有关。这些观察结果表明,BIN1 可以通过改变 Tau 的清除率并促进富含 Tau 的细胞外囊泡的释放,从而促进 AD 相关 Tau 病理学的进展。