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小鼠19号染色体三体中的行为和发育异常:染色体失衡诱发智力迟钝的动物模型。

Behavioural and developmental abnormalities in mouse trisomy 19: an animal model of mental retardation induced by chromosome imbalance.

作者信息

Buselmaier W, Bacchus C, Grohé G, Winking H

机构信息

Institut für Humangenetik und Anthropologie, Universität Heidelberg, Federal Republic of Germany.

出版信息

Teratology. 1988 Feb;37(2):167-74. doi: 10.1002/tera.1420370210.

Abstract

Murine trisomy 19 (Ts19) can be regarded as a general model of human trisomies. It is the only autosomal trisomy in the mouse that survives the perinatal period. Therefore, it is the only animal model available for postnatal investigations of trisomy-specific mental retardation. To evaluate the extent of developmental retardation during the late-embryonic and fetal period of gestation, total body weight development was documented for 60 Ts19-fetuses and compared with that of 219 euploid in utero-mates. In addition, a postnatal study on body-weight development of 77 Ts19-neonates and 74 euploid littermates was performed starting on day 1 postpartum and continuing until spontaneous death or until day 22. Forty-seven Ts19-individuals were further tested in nine behavioural test systems in order to determine their neurophysiological developmental profile. Findings were compared with age-dependent morphologic and physiologic parameters. The data obtained in the present study show a significant retardation of organ- and body-weight development in Ts19-mice starting on day 14 of gestation. Retardation of physiological parameters is progressive and persists throughout the perinatal and postnatal periods. Furthermore, the trisomic individuals showed specific behavioural abnormalities.

摘要

小鼠19号染色体三体(Ts19)可被视为人类三体的通用模型。它是小鼠中唯一能在围产期存活的常染色体三体。因此,它是唯一可用于出生后三体特异性智力发育迟缓研究的动物模型。为了评估妊娠晚期胚胎和胎儿期发育迟缓的程度,记录了60只Ts19胎儿的总体重发育情况,并与219只同窝正常二倍体胎儿进行比较。此外,对77只Ts19新生小鼠和74只同窝正常二倍体小鼠进行了出生后体重发育研究,从出生后第1天开始,持续到自然死亡或直到第22天。对47只Ts19个体在九个行为测试系统中进行了进一步测试,以确定它们的神经生理发育特征。研究结果与年龄相关的形态学和生理学参数进行了比较。本研究获得的数据表明,Ts19小鼠从妊娠第14天开始,器官和体重发育明显迟缓。生理参数的迟缓是渐进性的,在围产期和出生后阶段持续存在。此外,三体个体表现出特定的行为异常。

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