Role and Regulation of Copper and Zinc Transport Proteins in the Central Nervous System

  • C. W. Levenson
  • N. M. Tassabehji
Reference work entry


The trace elements copper and zinc are essential for the molecular and physiological functions of the central nervous system (CNS). These cations act as cofactors for enzymes that regulate every aspect of CNS function, including neuronal development and plasticity, neurotransmitter synthesis and processing, cellular metabolism and energy production, and gene expression. Imbalances in zinc have been associated with a variety of clinical disorders, including Alzheimer's disease (AD) and Parkinson's disease. Zinc has also been implicated in the neuronal damage and death associated with ischemia, seizure disorders, and brain trauma. Genetic disorders characterized by copper toxicity and copper deficiency have severe neurological consequences. Thus, it is important that subcellular copper and zinc balance be maintained precisely. This task is accomplished by several families of metal-specific transport proteins. For each metal, this chapter will begin with a brief introduction to the role of this metal in the CNS. This will be followed by the function and regulation of the known transport proteins involved in cellular uptake, intracellular trafficking, and cellular export in the CNS. There will also be discussions of the clinical implications of zinc and copper transporter abnormalities as well as the possible ways that an understanding of these transporters could lead to the development of new treatments for a variety of neurological disorders.


Zinc Deficiency Zinc Transporter Central Nervous System Function Free Zinc Cellular Zinc 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations:


Alzheimer's disease


acrodermatitis enteropathica


alpha‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid glutamate receptor


Menkes protein, MNK


Wilsons protein, WND


bovine spongiform encephalopathy




cytochrome c oxidase


copper chaperone for SOD


Creutzfeldt‐Jakob disease


central nervous system






dopamine beta monooxygenase


divalent metal transporter‐1


FK506‐binding protein 52


gamma‐aminobutyric acid


GABAA receptor




gonadotropin‐releasing hormone




LIV‐1 subfamily of ZIP zinc transporters


matrix metalloproteinase protein


Menkes protein, ATP7A


melanocortin stimulating hormone




N‐methyl‐d‐aspartate glutamate receptor


neuropeptide Y


nuclear respiratory factor


peptidylglycine α‐amidating monooxygenase


superoxide dismutase


trans‐Golgi network


thyrotropin‐releasing hormone


Voltage‐gated calcium channels


vasoactive intestinal polypeptide


Wilsons disease protein, APT7B


Zrt/Irt‐like proteins




zinc transporter


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© Springer Science+Business Media, LLC. 2007

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  • C. W. Levenson
  • N. M. Tassabehji

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