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首页> 外文期刊>American journal on intellectual and developmental disabilities: AJIDD >Advancing Knowledge of Down Syndrome Brain Development and Function With Human Stem Cells
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Advancing Knowledge of Down Syndrome Brain Development and Function With Human Stem Cells

机译：促进唐氏综合征脑发育的知识和人干细胞功能

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Our bodies are made up of over 250 specific cell types, and all initially arise from stem cells during embryonic development. Stem cells have two characteristics that make them unique: (1) they are pluripotent, meaning that they can differentiate into all cell types of the body, and (2) they are capable of self-renewal to generate more of themselves and are thus able to populate an organism. Human pluripotent stem cells were first isolated from human embryos twenty years ago (Thomson et al., 1998) and more recently, technology to reprogram somatic cells, such as skin and blood, to induced pluripotent stem cells has emerged (Park et al., 2008; Takahashi et al., 2007; Yu et al., 2007). Induced pluripotent stem cells, or iPSCs, arc particularly valuable as disease specific iPSCs can be generated from individuals with specific genetic mutations diseases. Researchers have harnessed the power of stem cells to understand many aspects of developmental biology in model organisms (e.g. worms, mice) and more recently, in humans. Human stem cells in culture recapitulate development. For example, formation of the brain occurs prenatally and follows a specific pattern of timing and cell generation. Human stem cells in the culture dish follow a similar pattern when exposed to developmental cues and can thus be used to understand aspects of prenatal human brain development that are not accessible by other means. Disease-specific iPSCs are a valuable tool to model neural development in specific neurodevelopmental disorders like Down syndrome. Down syndrome is a classic developmental disorder; mistakes that are made during development of a particular organ system result in the characteristics of the disorder. In the brain, mistakes during prenatal brain development lead to intellectual disability. Trisomy 21 (Ts21) iPSCs generated from somatic cells of Down syndrome individuals may enable us to understand the mistakes made during Down syndrome brain development.
机译：我们的身体由250多种特异性细胞类型组成，最初在胚胎发育期间从干细胞产生。干细胞具有两个特征，使它们独特：（1）它们是多能的，这意味着它们可以区分为身体的所有细胞类型，并且（2）它们能够自更新以产生更多的自我，因此可以填充生物体。二十年前首先从人胚胎中分离出人多能干细胞（Thomson等，1998），更近，出现了诱导多能干细胞的重新编程体细胞的技术（如皮肤和血液）（Park等人， 2008; Takahashi等，2007; Yu等，2007）。诱导多能干细胞或IPSC，弧形弧特别有价值，因为疾病特异性IPSC可以从具有特异性遗传突变疾病的个体产生。研究人员利用干细胞的力量，了解模型生物（例如蠕虫，小鼠）和最近在人类中的发展生物学的许多方面。培养物中的人干细胞概括地培养发育。例如，大脑的形成是处理的，并遵循特定的定时和细胞产生。在暴露于发育线索时，培养皿中的人干细胞在培养皿中遵循类似的模式，因此可以用于理解不可通过其他方式访问的产前人脑发育的方面。特定疾病的IPSC是一种有价值的工具，可以在特异性神经发育障碍中模拟神经发育，如下综合征。唐氏综合症是一种经典的发育障碍;在特定器官系统开发期间制造的错误导致疾病的特征。在大脑中，产前脑发育过程中的错误导致智力残疾。从唐氏综合征个人的体细胞生成的三兆癣21（TS21）IPSC可以使我们了解在唐氏综合症脑发育过程中所做的错误。

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