Metal Storage Disorders: Inherited Disorders of Copper and Manganese Metabolism and Movement Disorders

Chapter 17 - Metal Storage Disorders: Inherited Disorders of Copper and Manganese Metabolism and Movement Disorders

from Section II - A Metabolism-Based Approach to Movement Disorders and Inherited Metabolic Disorders

Published online by Cambridge University Press: 24 September 2020

Karin Tuschl and

Peter T. Clayton

Edited by

Darius Ebrahimi-Fakhari and

Phillip L. Pearl

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Darius Ebrahimi-Fakhari: Affiliation:
Harvard Medical School
Phillip L. Pearl: Affiliation:
Harvard Medical School

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Summary

Copper is one of the six transition metals that have important biochemical roles in humans, particularly in catalysis and electron transport [1, 2]. Because it can exist in two redox states (Cu2+/Cu+), it can participate in redox reactions involving transfer of an electron, but if it builds up it can also generate potentially toxic reactive oxygen species by Fenton chemistry. Examples of copper in redox enzymes include: complex IV of the mitochondrial respiratory chain, copper–zinc superoxide dismutase, ceruloplasmin (ferroxidase), lysyl oxidase, dopamine beta-hydroxylase, and tyrosinase.

Type: Chapter
Information: Movement Disorders and Inherited Metabolic Disorders
Recognition, Understanding, Improving Outcomes
, pp. 230 - 243

DOI: https://doi.org/10.1017/9781108556767 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2020

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