Mammalian copper-transporting P-type ATPases, ATP7A and ATP7B: emerging roles.

Mammalian copper-transporting P-type ATPases, ATP7A and ATP7B: emerging roles.
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DOI:
10.1016/j.biocel.2009.11.007
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发表时间:
2010-02
影响因子:
4
通讯作者:
Mercer, Julian F. B.
Mercer, Julian F. B.
中科院分区:
生物学2区
文献类型:
--
作者:
La Fontaine, Sharon;Ackland, M. Leigh;Mercer, Julian F. B.

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铜 (Cu) 在多种且数量不断增加的途径、生理和疾病过程中发挥作用。这些作用证明了铜在生物学中的根本重要性以及了解调节铜稳态机制的必要性。哺乳动物铜转运 P 型 ATP 酶 ATP7A 和 ATP7B 是调节体内铜状态的两种关键蛋白。它们将铜转运穿过细胞膜以实现生物合成和保护功能,使铜能够发挥其作为许多必需酶的结构辅因子的作用,并防止细胞内铜的毒性积聚。多种调节机制在转录和翻译后水平上发挥作用,以确保生理和病理生理过程有足够的铜供应。本综述总结了最近的文献,这些文献揭示了 Cu-ATP 酶在健康和疾病中的新作用。
Copper (Cu) has a role in a diverse and increasing number of pathways, physiological and disease processes. These roles are testament to the fundamental importance of Cu in biology and the need to understand the mechanisms that regulate Cu homeostasis. The mammalian Cu-transporting P-type ATPases ATP7A and ATP7B are two key proteins that regulate the Cu status of the body. They transport Cu across cellular membranes for biosynthetic and protective functions, enabling Cu to fulfill its role as a structural cofactor for many essential enzymes, and to prevent a toxic build-up of Cu inside cells. A variety of regulatory mechanisms operate at transcriptional and post-translational levels to ensure adequate Cu supplies for both physiological and pathophysiological processes. This review summarizes the recent literature that is revealing the emerging roles of the Cu-ATPases in health and disease.
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