Biogen, Cambridge, MA, United States of America.
Department of Cell Biology, Harvard Medical School, Boston, MA, United States of America.
PLoS One. 2019 Aug 13;14(8):e0220125. doi: 10.1371/journal.pone.0220125. eCollection 2019.
BIN1 is the most important risk locus for Late Onset Alzheimer's Disease (LOAD), after ApoE. BIN1 AD-associated SNPs correlate with Tau deposition as well as with brain atrophy. Furthermore, the level of neuronal-specific BIN1 isoform 1 protein is decreased in sporadic AD cases in parallel with neuronal loss, despite an overall increase in BIN1 total mRNA. To address the relationship between reduction of BIN1 and neuronal cell loss in the context of Tau pathology, we knocked-down endogenous murine Bin1 via stereotaxic injection of AAV-Bin1 shRNA in the hippocampus of mice expressing Tau P301S (PS19). We observed a statistically significant reduction in the number of neurons in the hippocampus of mice injected with AAV-Bin1 shRNA in comparison with mice injected with AAV control. To investigate whether neuronal loss is due to deletion of Bin1 selectively in neurons in presence Tau P301S, we bred Bin1flox/flox with Thy1-Cre and subsequently with PS19 mice. Mice lacking neuronal Bin1 and expressing Tau P301S showed increased mortality, without increased neuropathology, when compared to neuronal Bin1 and Tau P301S-expressing mice. The loss of Bin1 isoform 1 resulted in reduced excitability in primary neurons in vitro, reduced neuronal c-fos expression as well as in altered microglia transcriptome in vivo. Taken together, our data suggest that the contribution of genetic variation in BIN1 locus to AD risk could result from a cell-autonomous reduction of neuronal excitability due to Bin1 decrease, exacerbated by the presence of aggregated Tau, coupled with a non-cell autonomous microglia activation.
BIN1 是继 ApoE 之后,导致晚发性阿尔茨海默病(LOAD)的最重要风险基因座。与 Tau 沉积以及脑萎缩相关的 BIN1 AD 相关 SNP 与神经元特异性 BIN1 同工型 1 蛋白水平降低有关,尽管总 BIN1 mRNA 增加,但在散发性 AD 病例中,这种蛋白水平平行于神经元丢失而降低。为了解 Tau 病理学背景下 BIN1 减少与神经元细胞丢失之间的关系,我们通过立体定向注射 AAV-Bin1 shRNA 在表达 Tau P301S 的 PS19 小鼠的海马体中敲低内源性鼠源 Bin1。与注射 AAV 对照的小鼠相比,注射 AAV-Bin1 shRNA 的小鼠海马体中的神经元数量呈统计学显著减少。为了研究神经元丢失是否由于 Tau P301S 存在时神经元中 Bin1 的选择性缺失,我们将 Bin1flox/flox 与 Thy1-Cre 以及随后与 PS19 小鼠进行了杂交。与神经元 Bin1 和 Tau P301S 表达的小鼠相比,缺乏神经元 Bin1 并表达 Tau P301S 的小鼠死亡率增加,而神经病理学没有增加。Bin1 同工型 1 的缺失导致体外原代神经元兴奋性降低、神经元 c-fos 表达减少以及体内小胶质细胞转录组改变。总之,我们的数据表明,BIN1 基因座的遗传变异对 AD 风险的贡献可能是由于 Bin1 减少导致神经元兴奋性的细胞自主性降低,加上聚集的 Tau 的存在,以及非细胞自主性小胶质细胞激活,从而导致的。