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节段性三体综合征作为唐氏综合征的小鼠模型。

Segmental trisomy as a mouse model for Down syndrome.

作者信息

Davisson M T, Schmidt C, Reeves R H, Irving N G, Akeson E C, Harris B S, Bronson R T

机构信息

Jackson Laboratory, Bar Harbor, Maine 04609.

出版信息

Prog Clin Biol Res. 1993;384:117-33.

PMID:8115398
Abstract

Mice trisomic for Chromosome (Chr) 16 have been used extensively as an animal model for human Down Syndrome (Trisomy 21). This system has drawbacks, however: trisomy for all of Chr 16 is incompatible with postnatal survival and produces trisomy for many more genes than those conserved in human Chr 21. We report here the development and preliminary characterization of mice that are trisomic for only the segment of mouse Chr 16 that is conserved in human Chr 21. While these segmentally trisomic mice, Ts(17(16)) 65Dn, do not appear to have all the features characteristic of Down Syndrome, they represent a mouse model that survives to adulthood and may be useful to study features of Down Syndrome that develop later in life, such as susceptibility to infection, increased incidence of leukemia, and Alzheimer-like neuropathology.

摘要

16号染色体三体的小鼠已被广泛用作人类唐氏综合征(21三体)的动物模型。然而,这个系统存在缺陷:整个16号染色体三体与出生后存活不兼容,并且产生三体的基因比人类21号染色体中保守的基因多得多。我们在此报告仅在人类21号染色体中保守的小鼠16号染色体片段三体的小鼠的培育及初步特征。虽然这些片段三体小鼠Ts(17(16)) 65Dn似乎并不具备唐氏综合征的所有特征,但它们代表了一种能存活至成年的小鼠模型,可能有助于研究唐氏综合征在生命后期出现的特征,如易感染性、白血病发病率增加以及阿尔茨海默病样神经病理学。

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