Copper–metallothionein in the Kidney of Macular Mice: A Model for Menkes Disease
Copper–metallothionein in the Kidney of Macular Mice: A Model for Menkes Disease
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黄斑小鼠肾脏中的铜金属硫蛋白：门克斯病模型
DOI：
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发表时间：
1997
影响因子：
3.2
通讯作者：
M. Kurasaki
中科院分区：
文献类型：
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作者：
M. Suzuki;M. Okabe;M. Kurasaki
Menkes病是一种X连锁的铜代谢紊乱。在该疾病的模型黄斑小鼠的肾脏中发现过量的铜，作为来自小鼠肾脏的铜金属硫蛋白（Cu-MT）。基于其自身荧光发射特性的Cu-MT的组织化学研究表明，该蛋白主要存在于皮质的近曲小管（PCT）细胞中。PCT细胞是重金属引起肾毒性的主要部位。MT mRNA也在皮层中观察到，表明该蛋白质在该区域生物合成。在这些结果的基础上，我们认为，铜MT的生物合成和降解反复发生在PCT细胞的皮质。我们还比较了Cu-MT在黄斑小鼠和Long-Evans肉桂大鼠（Wilson病模型）中的组织化学定位。这种比较的意义进行了讨论。（J Histochem Cytochem 45：1493-1501，1997）
Menkes disease is an X-linked disorder of copper metabolism. Excess amounts of copper in the kidney of Macular mice, a model for this disease, were found as copper–metallothionein (Cu–MT) from kidney of the mice. Histochemical studies of Cu–MT based on its autofluorescent emission properties showed that the protein was predominant in the proximal convoluted tubule (PCT) cells of the cortex. PCT cells are known to be the primary site of the nephrotoxicity caused by heavy metals. MT mRNA was also observed in the cortex, indicating that the protein was biosynthesized in this region. On the basis of these results, we suggest that biosynthesis and degradation of Cu–MT occur repeatedly in the PCT cells of the cortex. We also compared the histochemical localization of Cu–MT in Macular mice and Long–Evans cinnamon rats, a model for Wilson's disease. The significance of this comparison is discussed. (J Histochem Cytochem 45:1493–1501, 1997)
DOI：
10.1073/pnas.93.24.14030
发表时间：
1996-11-26
影响因子：
11.1
作者：
Yamaguchi, Y;Heiny, ME;Gitlin, JD
通讯作者：
Gitlin, JD