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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

By
Karin Tuschl  and
Peter T. Clayton
Edited by
Darius Ebrahimi-Fakhari  and
Phillip L. Pearl
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
Publisher: Cambridge University Press
Print publication year: 2020

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