Evaluation of the mouse mutant "wasted" as an animal model for ataxia telangiectasia. I. Age-dependent and tissue-specific effects.
Evaluation of the mouse mutant "wasted" as an animal model for ataxia telangiectasia. I. Age-dependent and tissue-specific effects.
复制标题
评估“废”的小鼠突变体作为共济失调毛细血管扩张的动物模型。
DOI：
10.1016/0027-5107(86)90102-8
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发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Shultz,LD
中科院分区：
文献类型：
--
作者：
Tezuka,H;Inoue,T;Noguti,T;Kada,T;Shultz,LD
本文研究了遗传性共济失调毛细血管扩张症（AT）的动物模型--废鼠的生物学、细胞遗传学和生化特性。在受影响的纯合子中，观察到脾脏和胸腺与体重的比率呈明显的年龄依赖性下降，肝脏与体重的比率轻微但显著下降，而肾脏中未发现这种变化。自发的和γ射线诱导的染色体畸变的频率中观察到一个年龄依赖性的增加浪费小鼠骨髓细胞。在同窝对照小鼠中，未观察到年龄依赖性的这些变化。据报道，AT细胞中缺乏的引物激活酶的活性也随着年龄的增长在脾细胞中下降，但在受影响小鼠的肝细胞中没有。然而，在脱嘌呤DNA内切酶活性的变化没有检测到的发育阶段检查。这些数据表明，这种小鼠突变体可以作为一个有用的动物模型，研究DNA修复和淋巴组织分化之间的关系。
The wasted mouse, an animal model proposed for the genetically transmitted human disease ataxia telangiectasia (AT), was examined for its biological, cytogenetic and biochemical properties. In affected homozygotes, a marked age-dependent decrease in the ratio of spleen and thymus to body weight, and a slight but significant decrease in the liver to body weight ratio were observed while no such change was found in the kidney. An age-dependent increase was observed in the frequency of both spontaneous and gamma-ray-induced chromosomal aberrations in bone marrow cells of wasted mice. In littermate control mice, neither of these alterations was observed in an age-dependent manner. The activity of a primer activating enzyme, which has been reported to be deficient in AT cells, also decreased with age in spleen cells, but not in liver cells of affected mice. However, alterations in apurinic DNA endonuclease activity were not detected in the developmental stages examined. These data indicate that this mouse mutant may serve as a useful animal model for studying the relationships between DNA repair and lymphoid tissue differentiation.