诱导多能干细胞
胚胎干细胞
神经科学
类有机物
神经退行性变
生物
干细胞
三体
表型
细胞生物学
计算生物学
基因
疾病
遗传学
医学
病理
作者
Gillian Gough,Niamh O’Brien,Ivan Alić,Pollyanna Goh,Yee Jie Yeap,Jürgen Groet,Dean Nižetić,Aoife Murray
出处
期刊:Progress in Brain Research
日期:2020-01-01
卷期号:: 55-90
被引量:14
标识
DOI:10.1016/bs.pbr.2019.10.003
摘要
Down Syndrome (DS) is a complex chromosomal disorder, with neurological issues, featuring among the symptoms. Primary neuronal cells and tissues are extremely useful, but limited both in supply and experimental manipulability. To better understand the cellular, molecular and pathological mechanisms involved in DS neurodevelopment and neurodegeneration, a range of different cellular models have been developed over the years including human: mouse hybrid cells, transchromosomic mouse embryonic stem cells (ESCs) and human ESC and induced pluripotent stem cells derived from different sources. All of these model systems have provided useful information in the study of DS. Furthermore, different technologies to genetically modify or correct trisomy of either single genes or the whole chromosome have been developed using these cellular models. New techniques and protocols to allow better modeling of cellular mechanisms and disease processes are being developed and the use of cerebral organoids offers great promise for future research into the neural phenotypes seen in DS.
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