Abnormal cellular copper metabolism in the blotchy mouse.
Abnormal cellular copper metabolism in the blotchy mouse.
复制标题
斑点小鼠细胞铜代谢异常。
DOI：
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发表时间：
1978
期刊：
影响因子：
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通讯作者：
C. H. Hill
中科院分区：
文献类型：
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作者：
B. Starcher;J. Madaras;D. Fisk;E. F. Perry;C. H. Hill
在 X 染色体上的斑驳基因座上带有斑点 (Moblo/y) 等位基因的雄性小鼠中，发现了铜代谢缺陷。肠道铜吸收量仅为正常小鼠的 64%，肝脏铜水平仅为对照组的 56%。铜蓝蛋白和心脏细胞色素c氧化酶活性正常，但培养的成纤维细胞的赖氨酰氧化酶活性仅为对照水平的45%。从这些突变体培养的成纤维细胞中铜的积累量是正常值的五倍。成纤维细胞中铜的积累与分子量约为 12,000 的蛋白质有关。
Defective copper metabolism was demonstrated in male mice bearing the blotchy (Moblo/y) allele at the mottled locus on the X-chromosome. Copper absorption from the gut was only 64% of that found in normal mice and hepatic copper levels were only 56% of the controls. Ceruloplasmin and heart cytochrome c oxidase activities were normal, yet lysyl oxidase activity from cultured fibroblasts was only 45% of control levels. Copper accumulated in fibroblasts cultured from these mutants to values that were five times normal. The accumulation of copper in the fibroblasts was associated with a protein of approximately 12,000 molecular weight.