Lane N J, Balbo A, Stoll J, Rapoport S I
Department of Zoology, University of Cambridge, UK.
Tissue Cell. 1994 Aug;26(4):477-88. doi: 10.1016/0040-8166(94)90001-9.
Murine trisomy 16 (Ts16) is an animal model for Down's syndrome (human trisomy 21), because mouse chromosome 16 is genetically homologous to human chromosome 21. Down's syndrome patients, older than 35 years, demonstrate the neuropathological and neurochemical defects characteristic of Alzheimer's disease and Ts16 mouse fetuses exhibit phenotypic abnormalities similar to those in Down's syndrome fetuses. Trisomic mouse fetuses, however, die in utero, and so do not survive long enough for their brains to develop the degenerative changes of aging. This can be overcome by grafting the fetal Ts16 hippocampus (an early site for the development of the pathological features characteristic of Alzheimer's disease), into the cerebral ventricle or striatum of a normal adult mouse host. We have made such transplants, which have survived for up to 12 months. Examining these grafts ultrastructurally at various stages from 1 to 12 months, and comparing them with normal control grafts, reveals no structural difference that could be deemed characteristic of Alzheimer disease; no neurofibrillary tangle or senile plaque was observed. These observations, together with the normal structure of the neuronal organelles in trisomic hippocampal tissue, suggest that trisomic mouse grafts are not a useful model for Alzheimer's disease, despite earlier reports to the contrary.
小鼠16三体综合征(Ts16)是唐氏综合征(人类21三体综合征)的动物模型,因为小鼠的16号染色体与人类的21号染色体在基因上具有同源性。35岁以上的唐氏综合征患者表现出阿尔茨海默病特有的神经病理学和神经化学缺陷,而Ts16小鼠胎儿表现出与唐氏综合征胎儿相似的表型异常。然而,三体小鼠胎儿在子宫内死亡,因此存活时间不足以使其大脑发生衰老的退行性变化。通过将Ts16胎儿海马体(阿尔茨海默病特征性病理特征早期出现的部位)移植到正常成年小鼠宿主的脑室或纹状体中,可以克服这一问题。我们已经进行了这样的移植,移植体存活了长达12个月。在1至12个月的不同阶段对这些移植体进行超微结构检查,并将它们与正常对照移植体进行比较,结果显示没有可被视为阿尔茨海默病特征的结构差异;未观察到神经原纤维缠结或老年斑。这些观察结果,连同三体海马组织中神经元细胞器的正常结构,表明三体小鼠移植体并非阿尔茨海默病的有用模型,尽管早期有相反的报道。