Copper metabolism in the macular mutant mouse: an animal model of Menkes's kinky-hair disease.
Copper metabolism in the macular mutant mouse: an animal model of Menkes's kinky-hair disease.
复制标题
黄斑突变小鼠的铜代谢：门克斯卷发病的动物模型。
DOI：
10.1159/000242849
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
T. Taguchi
中科院分区：
文献类型：
--
作者：
N. Shiraishi;K. Aono;T. Taguchi
测定了突变型（半合子黄斑雄性和纯合子黄斑雌性）、杂合子黄斑雌性和正常小鼠的组织铜含量。与正常小鼠相比，7日龄突变体和杂合子小鼠的肾脏和小肠中铜含量极高，而其他组织（肝、脑、脾和血清）中铜含量较低。与正常小鼠相比，除了妊娠14天的突变体胎儿外，突变体小鼠全身的铜含量在三个阶段（妊娠18天、1天大和7天大）都极低。与正常小鼠相比，在妊娠18天的突变体胎儿和在1天的突变体中的肾铜含量没有改变，而在7和14天的突变体小鼠中的肾铜含量显著升高。与正常小鼠相比，突变小鼠的肝脏铜含量在所有阶段都极低。铜疗法应用于7日龄的突变小鼠。注射后第1天，突变体小鼠的肝脏铜含量与正常对照小鼠相比略有增加，而肾脏铜含量则显著增加。在注射后7天，突变体中的肝铜含量与正常对照小鼠相比大大降低，而肾铜含量的增加仍然存在。
The tissue copper contents were measured in mutant (hemizygous macular male and homozygous macular female), heterozygous macular female and normal mice. The copper content in kidney and small intestine from 7-day-old mutant and heterozygote were extremely high compared to normal mice, whereas the copper content in other tissues (liver, brain, spleen and serum) was low. Copper content in whole body of mutant mice was extremely low at three stages (18 days gestation, 1 day old, and 7 days old) compared to normal mice with the exception of the mutant fetus at 14 days of gestation. Renal copper contents in the mutant fetus at 18 days of gestation and in the 1-day-old mutant were not changed compared to normal mice, whereas that in 7- and 14-day-old mutant mice was significantly elevated. Hepatic copper content of the mutant mice was extremely low at all stages compared to normal mice. Copper therapy was applied to 7-day-old mutant mice. At 1 day after injection, hepatic copper content in the mutants had increased slightly in comparison with the normal control mice, whereas renal copper content was extremely increased. At 7 days after injection, hepatic copper content in the mutants was decreased greatly in comparison with normal control mice, whereas an increase in renal copper content had remained.