Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation
Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation
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DOI：
10.1016/j.bbr.2010.05.010
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发表时间：
2010-12-01
影响因子：
2.7
通讯作者：
Berman, Robert F.
中科院分区：
文献类型：
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作者：
Hunsaker, Michael R.;Goodrich-Hunsaker, Naomi J.;Berman, Robert F.
脆性X预突变是FMR1基因上的串联CGG三核苷酸重复扩增，长度在55到200之间。已经开发出一种CGG敲入（CGG KI）小鼠，其CGG重复长度在70到350之间，并用于表征脆性X前兆突变携带者的组织病理学和认知缺陷。先前的研究表明，CGG - Kt小鼠在处理空间信息方面表现出进行性缺陷。为了进一步表征脆性X预突变中的认知缺陷，我们评估了CGG敲入（CGG KI）小鼠的时间顺序。研究人员测试了雌性CGG - KI小鼠记住两个物体呈现的时间顺序的能力。结果表明，在48周龄时，CGG重复扩增在150 ~ 200次之间的雌性CGG KI小鼠在时间顺序测试中的表现比野生型小鼠差，而CGG重复扩增在80 ~ 100次之间的雌性CGG Kt小鼠的表现与野生型小鼠相似。没有老鼠在发现新物体的存在方面有任何困难。这些数据表明雌性CGG KI小鼠在时间顺序上表现出CGG重复长度敏感缺陷。(C) 2010 Elsevier B.V.版权所有
The fragile X premutation is a tandem CGG trinucleotide repeat expansion on the FMR1 gene between 55 and 200 repeats in length. A CGG knock-in (CGG KI) mouse with CGG repeat lengths between 70 and 350 has been developed and used to characterize the histopathology and cognitive deficits reported in carriers of the fragile X premutation. Previous studies have shown that CGG Kt mice show progressive deficits in processing spatial information. To further characterize cognitive deficits in the fragile X premutation, temporal ordering in CGG knock-in (CGG KI) mice was evaluated. Female CGG KI mice were tested for their ability to remember the temporal order in which two objects were presented. The results demonstrate that at 48 weeks of age, female CGG KI mice with CGG repeat expansions between 150 and 200 CGG repeats performed more poorly on tests of temporal order than wildtype mice, whereas female CGG Kt mice with between 80 and 100 CGG repeats performed similarly to wildtype mice. No mice had any difficulty in detecting the presence of a novel object. These data suggest female CGG KI mice show a CGG repeat length-sensitive deficit for temporal ordering. (C) 2010 Elsevier B.V. All rights reserved.