Mouse models of cognitive disorders in trisomy 21:: A review
Mouse models of cognitive disorders in trisomy 21:: A review
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DOI：
10.1007/s10519-006-9056-9
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发表时间：
2006-05-01
影响因子：
2.6
通讯作者：
Soumireu-Mourat, Bernard
中科院分区：
文献类型：
--
作者：
Seregaza, Zohra;Roubertoux, Pierre L.;Soumireu-Mourat, Bernard
21三体(TRS21)是导致智力低下的最常见的遗传原因。虽然已知HSA21的一个额外拷贝的存在是该综合征的起因，但我们不知道哪225个HSA21基因对认知过程有影响。利用HSA21和MMU16、MMU10和MMU17之间的融合建立了TRS21的小鼠模型。现有的小鼠模型携带额外的MMU16或HSA21片段，覆盖全部HSA21(嵌合HSA21)或MMU16(TS16)；一些携带MMU16的大部分(Ts65Dn，Ts1Cje，Ms1Cje)，而另一些模型减少了覆盖D21S17-ETS2区域的连续片段或单个转基因基因。这为破译认知(学习、记忆和探索)和涉及每个染色体区域的相关大脑异常提供了一种巢设计策略。这篇综述证实了包含16个基因的D21S17-ETS2区域对认知障碍的关键但不是唯一的贡献。
Trisomy 21 (TRS21) is the most frequent genetic cause of mental retardation. Although the presence of an extra copy of HSA21 is known to be at the origin of the syndrome, we do not know which 225 HSA21 genes have an effect on cognitive processes. Mouse models of TRS21 have been developed using syntenies between HSA21 and MMU16, MMU10 and MMU17. Available mouse models carry extra fragments of MMU16 or of HSA21 that cover all of HSA21 (chimeric HSA21) or MMU16 (Ts16); some carry large parts of MMU16 (Ts65Dn, Ts1Cje, Ms1Cje), while others have reduced contiguous fragments covering the D21S17-ETS2 region or single transfected genes. This offers a nest design strategy for deciphering cognitive (learning, memory and exploration) and associated brain abnormalities involving each of these chromosomal regions. This review confirms the crucial but not exclusive contribution of the D21S17-ETS2 region encompassing 16 genes to cognitive disorders.