Lane Alicia R, Roberts Blaine R, Fahrni Christoph J, Faundez Victor
Department of Cell Biology, Emory University, 615 Michael St, Atlanta, GA 30322, USA.
Department of Biochemistry, Emory University, 1510 Clifton Rd, Atlanta, GA 30322, USA; Department of Neurology, Emory University, 12 Executive Park Dr NE, Atlanta, GA 30322, USA.
Neurobiol Dis. 2025 Aug;212:106974. doi: 10.1016/j.nbd.2025.106974. Epub 2025 May 23.
This primer aims to expose scientists who study the brain to the field of copper biology. We briefly discuss key copper homeostasis mechanisms and proteins and place these functions in the context of the brain and neurodevelopment. A small number of key copper genes are explored as representative examples of the importance of this metal to the brain. We show that these genes are expressed throughout the brain and their defects are linked to a diverse array of neurological phenotypes, which we discuss further in the context of several neurological and neurodegenerative diseases associated with dysregulation of copper. This review aims to expose interested scientists to the fundamental roles for copper in the brain, the primary proteins responsible for maintaining copper homeostasis in the brain, and the classic neurological diseases associated with this metal.
本入门读物旨在让研究大脑的科学家了解铜生物学领域。我们简要讨论了关键的铜稳态机制和蛋白质,并将这些功能置于大脑和神经发育的背景下。我们探讨了少数关键的铜基因,作为这种金属对大脑重要性的代表性例子。我们表明,这些基因在整个大脑中表达,它们的缺陷与一系列不同的神经表型有关,我们将在与铜失调相关的几种神经和神经退行性疾病的背景下进一步讨论这些表型。这篇综述旨在让感兴趣的科学家了解铜在大脑中的基本作用、负责维持大脑铜稳态的主要蛋白质,以及与这种金属相关的经典神经疾病。