Dept. of Psychology, Cornell University, Ithaca, NY, USA.
Neurobiol Dis. 2013 Oct;58:92-101. doi: 10.1016/j.nbd.2013.04.016. Epub 2013 Apr 30.
In addition to intellectual disability, individuals with Down syndrome (DS) exhibit dementia by the third or fourth decade of life, due to the early onset of neuropathological changes typical of Alzheimer's disease (AD). Deficient ontogenetic neurogenesis contributes to the brain hypoplasia and hypocellularity evident in fetuses and children with DS. A murine model of DS and AD (the Ts65Dn mouse) exhibits key features of these disorders, notably deficient ontogenetic neurogenesis, degeneration of basal forebrain cholinergic neurons (BFCNs), and cognitive deficits. Adult hippocampal (HP) neurogenesis is also deficient in Ts65Dn mice and may contribute to the observed cognitive dysfunction. Herein, we demonstrate that supplementing the maternal diet with additional choline (approximately 4.5 times the amount in normal rodent chow) dramatically improved the performance of the adult trisomic offspring in a radial arm water maze task. Ts65Dn offspring of choline-supplemented dams performed significantly better than unsupplemented Ts65Dn mice. Furthermore, adult hippocampal neurogenesis was partially normalized in the maternal choline supplemented (MCS) trisomic offspring relative to their unsupplemented counterparts. A significant correlation was observed between adult hippocampal neurogenesis and performance in the water maze, suggesting that the increased neurogenesis seen in the supplemented trisomic mice contributed functionally to their improved spatial cognition. These findings suggest that supplementing the maternal diet with additional choline has significant translational potential for DS.
除了智力障碍,唐氏综合征(DS)患者在生命的第三或第四个十年会出现痴呆,这是由于阿尔茨海默病(AD)的神经病理学变化典型的早期发病。未成熟的神经发生不足导致 DS 胎儿和儿童的大脑发育不良和细胞减少。唐氏综合征和 AD 的一种鼠模型(Ts65Dn 小鼠)表现出这些疾病的关键特征,特别是未成熟的神经发生不足、基底前脑胆碱能神经元(BFCN)退化以及认知缺陷。成年海马体(HP)神经发生在 Ts65Dn 小鼠中也不足,可能导致观察到的认知功能障碍。在此,我们证明,在母体饮食中补充额外的胆碱(大约是正常啮齿动物饲料中胆碱含量的 4.5 倍)可以显著改善成年三体小鼠在放射臂水迷宫任务中的表现。补充胆碱的 Ts65Dn 后代的表现明显优于未补充的 Ts65Dn 小鼠。此外,相对于未补充的对照,母体补充胆碱(MCS)的三体后代的成年海马体神经发生部分得到了正常化。在水迷宫中的表现与成年海马体神经发生之间存在显著相关性,表明补充的三体小鼠中增加的神经发生对其空间认知能力的提高有功能上的贡献。这些发现表明,母体饮食中补充额外的胆碱对 DS 具有重要的转化潜力。