Alt E R, Sternlieb I, Goldfischer S
Department of Pathology, Albert Einstein College of Medicine, Bronx, New York 10461.
Int Rev Exp Pathol. 1990;31:165-88. doi: 10.1016/b978-0-12-364931-7.50011-2.
Researchers have been able to demonstrate the cytotoxicity of copper overload in animal models. This has allowed them to not only localize the intracellular distribution of copper but also to study directly the subsequent organelle injury at the ultrastructural level. The lesions seen in copper overload appear to vary from species to species. In humans, marked mitochondrial abnormalities are seen in Wilson's disease while diet overloaded rats show nuclear destruction and various membranous abnormalities. Sequestration of copper within lysosomes appears to protect hepatocytes from its toxicity. However, the mechanism by which the metal is incorporated into lysosomes is not known.
研究人员已能够在动物模型中证明铜过载的细胞毒性。这使他们不仅能够确定铜在细胞内的分布，还能在超微结构水平上直接研究随后的细胞器损伤。铜过载中出现的病变似乎因物种而异。在人类中，威尔逊病可见明显的线粒体异常，而饮食过量的大鼠则表现出核破坏和各种膜异常。铜在溶酶体内的隔离似乎能保护肝细胞免受其毒性。然而，金属被纳入溶酶体的机制尚不清楚。
