TOXIC MILK, A NEW MUTATION AFFECTING COPPER-METABOLISM IN THE MOUSE
TOXIC MILK, A NEW MUTATION AFFECTING COPPER-METABOLISM IN THE MOUSE
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DOI：
10.1093/oxfordjournals.jhered.a109751
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发表时间：
1983-01-01
影响因子：
3.1
通讯作者：
RAUCH, H
中科院分区：
文献类型：
--
作者：
RAUCH, H
毒牛奶，tx，一种新的常染色体隐性突变小鼠的描述。由突变雌性产生的窝仔显示出包括生长不良、色素减退、震颤和最终在2周龄时死亡的综合征。这些特点，符合铜缺乏症，是由于失败的妊娠肝铜积累加剧了生存的牛奶大大降低铜含量。这样的婴儿可以通过正常母鼠的寄养或补充铜的管理来挽救。突变体本身积累了非常高浓度的肝脏铜，最终导致肝脏疾病。血浆铜蓝蛋白活性降低进一步证明了肝脏铜代谢异常。
Toxic milk, tx, a new autosomal recessive mutation in mice is described. Litters produced by mutant females display a syndrome including poor growth, hypopigmentation, tremors and ultimately death at 2 wk of age. These features, consistent with Cu deficiency, are attributed to failure in gestational hepatic Cu accumulation exacerbated by subsistence on milk with greatly reduced Cu content. Such infants can be rescued by foster-nursing on normal dams or by administration of supplemental Cu. Mutants themselves amass extraordinarily large concentrations of hepatic Cu that ultimately leads to liver disease. Erroneous hepatic Cu metabolism is further evidenced by reduced ceruloplasmin activity.