Copper and prion disease
Copper and prion disease
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DOI：
10.1016/s0361-9230(01)00453-1
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发表时间：
2001-05-15
影响因子：
3.8
通讯作者：
Brown, DR
中科院分区：
文献类型：
--
作者：
Brown, DR
Prion蛋白是一种由神经元表达的细胞表面糖蛋白。它的功能一直难以捉摸，直到最近被证明是一种铜结合蛋白。现在有强有力的证据表明，Prion蛋白在正常的大脑铜代谢中起到了作用。Prion蛋白的表达改变了细胞对铜的摄取，并增强了铜对超氧化物歧化酶的掺入。此外，蛋白本身也可以作为一种超氧化物歧化酶。Prion病的一个方面是将有功能的Prion蛋白转化为聚集的淀粉样蛋白。这种转换可能会改变普里恩蛋白的功能或使其消失。这些结果表明，Pron病可能涉及到脑铜稳态的紊乱。(C)2001年爱思唯尔科学公司。
The prion protein is a cell surface glyco-protein expressed by neurones. Its function has remained elusive until it was recently shown to be a copper binding protein. There is now strong evidence that the prion protein has a role in normal brain copper metabolism. Prion protein expression alters copper uptake into cells and enhances copper incorporation into superoxide dismutase. Furthermore the prion protein itself can act as a superoxide dismutase. One aspect of prion disease is the conversion of functional prion protein into an aggregated amyloid. This conversion may alter the function of the prion protein or abolish it. These results suggest that prion disease may involve disturbance to brain copper homeostasis. (C) 2001 Elsevier Science Inc.