Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY, United States of America.
Department of Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY, United States of America.
PLoS One. 2018 Apr 10;13(4):e0195771. doi: 10.1371/journal.pone.0195771. eCollection 2018.
The microtubule associated protein tau plays a critical role in the pathogenesis of neurodegenerative disease. Recent studies suggest that tau also plays a role in disorders of neuronal connectivity, including epilepsy and post-traumatic stress disorder. Animal studies have shown that the MAPT gene, which codes for the tau protein, undergoes complex pre-mRNA alternative splicing to produce multiple isoforms during brain development. Human data, particularly on temporal and regional variation in tau splicing during development are however lacking. In this study, we present the first detailed examination of the temporal and regional sequence of MAPT alternative splicing in the developing human brain. We used a novel computational analysis of large transcriptomic datasets (total n = 502 patients), quantitative polymerase chain reaction (qPCR) and western blotting to examine tau expression and splicing in post-mortem human fetal, pediatric and adult brains. We found that MAPT exons 2 and 10 undergo abrupt shifts in expression during the perinatal period that are unique in the canonical human microtubule-associated protein family, while exon 3 showed small but significant temporal variation. Tau isoform expression may be a marker of neuronal maturation, temporally correlated with the onset of axonal growth. Immature brain regions such as the ganglionic eminence and rhombic lip had very low tau expression, but within more mature regions, there was little variation in tau expression or splicing. We thus demonstrate an abrupt, evolutionarily conserved shift in tau isoform expression during the human perinatal period that may be due to tau expression in maturing neurons. Alternative splicing of the MAPT pre-mRNA may play a vital role in normal brain development across multiple species and provides a basis for future investigations into the developmental and pathological functions of the tau protein.
微管相关蛋白 tau 在神经退行性疾病的发病机制中起着关键作用。最近的研究表明,tau 还在神经元连接障碍中发挥作用,包括癫痫和创伤后应激障碍。动物研究表明,编码 tau 蛋白的 MAPT 基因在大脑发育过程中通过复杂的前体 mRNA 选择性剪接产生多种异构体。然而,人类数据,特别是在发育过程中 tau 剪接的时间和区域变化方面的数据却很缺乏。在这项研究中,我们首次详细检查了发育中的人类大脑中 MAPT 选择性剪接的时间和区域顺序。我们使用了一种新的计算分析方法,对大型转录组数据集(总 n = 502 例患者)、定量聚合酶链反应(qPCR)和蛋白质印迹进行了分析,以检查死后人类胎儿、儿科和成人大脑中的 tau 表达和剪接情况。我们发现,MAPT 外显子 2 和 10 在围产期的表达发生了突然的转变,这在经典的微管相关蛋白家族中是独一无二的,而外显子 3 则表现出微小但显著的时间变化。tau 异构体的表达可能是神经元成熟的标志,与轴突生长的开始时间相关。不成熟的脑区,如神经节隆起和菱形唇,tau 表达非常低,但在更成熟的区域,tau 表达或剪接的变化很小。因此,我们在人类围产期期间证明了 tau 异构体表达的突然、进化上保守的转变,这可能是由于成熟神经元中的 tau 表达所致。MAPT 前体 mRNA 的选择性剪接可能在多种物种的正常大脑发育中起着至关重要的作用,并为未来研究 tau 蛋白的发育和病理功能提供了基础。