Sago H, Carlson E J, Smith D J, Kilbridge J, Rubin E M, Mobley W C, Epstein C J, Huang T T
Department of Pediatrics, University of California, Box 0546, San Francisco, CA 94143-0546, USA.
Proc Natl Acad Sci U S A. 1998 May 26;95(11):6256-61. doi: 10.1073/pnas.95.11.6256.
A mouse model for Down syndrome, Ts1Cje, has been developed. This model has made possible a step in the genetic dissection of the learning, behavioral, and neurological abnormalities associated with segmental trisomy for the region of mouse chromosome 16 homologous with the so-called "Down syndrome region" of human chromosome segment 21q22. Tests of learning in the Morris water maze and assessment of spontaneous locomotor activity reveal distinct learning and behavioral abnormalities, some of which are indicative of hippocampal dysfunction. The triplicated region in Ts1Cje, from Sod1 to Mx1, is smaller than that in Ts65Dn, another segmental trisomy 16 mouse, and the learning deficits in Ts1Cje are less severe than those in Ts65Dn. In addition, degeneration of basal forebrain cholinergic neurons, which was observed in Ts65Dn, was absent in Ts1Cje.
已经建立了一种唐氏综合征小鼠模型,即Ts1Cje。该模型使得对与小鼠16号染色体上与人类21号染色体片段21q22所谓的“唐氏综合征区域”同源区域的节段三体相关的学习、行为和神经异常进行基因剖析成为可能。在莫里斯水迷宫中的学习测试和自发运动活动评估揭示了明显的学习和行为异常,其中一些表明海马功能障碍。Ts1Cje中从Sod1到Mx1的重复区域比另一种16号染色体节段三体小鼠Ts65Dn中的小,并且Ts1Cje中的学习缺陷比Ts65Dn中的轻。此外,在Ts65Dn中观察到的基底前脑胆碱能神经元变性在Ts1Cje中未出现。