Copper Metabolism and the Liver
Copper plays a critical biochemical role in the function of many enzymes and proteins that contain this essential element. Consequently, copper deficiency leads to loss of the catalytic function of copper-dependent enzymes and structural changes in proteins with prosthetic copper, while excess metal is toxic and leads to cell injury and death. Homeostatic control mechanisms have evolved to coordinate a healthy balance for copper, allowing cells to accumulate sufficient copper to support essential biochemical reactions and yet prevent toxicity due to an excess of this metal. Much of our knowledge of these metabolic pathways is derived from the study of the inherited metabolic disorders of copper metabolism and from microbial and mouse models that have deciphered new copper transporters, metallochaperones, and signaling pathways. This in turn has led to a better understanding of the pathophysiology of each of the metabolic disorders of copper metabolism, aiding in diagnosis and treatment and in the design of future therapies. © 2009, John Wiley & Sons Ltd.
Start / End Page
Digital Object Identifier (DOI)