Genes regulating copper metabolism

The metabolism of Cu is intimately linked with its nutrition. From gut to enzymes, Cu bioavailability to key enzymes and other components operates through a complex mechanism that uses transport proteins as well as small molecular weight ligands. Steps in Cu transport through the blood, absorption b...

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Bibliographic Details
Published in:Molecular and cellular biochemistry 1998-11, Vol.188 (1-2), p.57-62
Main Authors: Harris, E D, Qian, Y, Reddy, M C
Format: Article
Language:English
Subjects:
Adenosine Triphosphatases - genetics
Adenosinetriphosphatase
Animals
Bioavailability
Caco-2 Cells
Carrier Proteins - genetics
Cation Transport Proteins
Cloning
Copper
Copper - metabolism
Copper - physiology
Copper-transporting ATPases
Genes
Genes - physiology
Humans
Mammals
Molecular weight
Recombinant Fusion Proteins
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Summary:The metabolism of Cu is intimately linked with its nutrition. From gut to enzymes, Cu bioavailability to key enzymes and other components operates through a complex mechanism that uses transport proteins as well as small molecular weight ligands. Steps in Cu transport through the blood, absorption by cells, and incorporation into enzymes are slowly being understood. Cloning and sequencing of the genes for Menkes disease and Wilson disease has shown that membrane-bound enzymes analogous to Cu-ATPases in prokaryotes are equally important to Cu transport and homeostasis in mammalian cells. The primary structure of the mammalian Cu-ATPases has been deduced from cDNAs from tissues and organs. It now appears that mammalian Cu-ATPase have tissue and developmental specificity. In this review, we will focus on the Cu-ATPase that has been identified with Menkes disease. An emphasis will be placed on the existence of multiple forms of the ATPase and some indication as to how the different isoforms befit their role in the normal physiology of copper, specifically transmembrane transport and maintenance of a favorable internal cellular environment.
ISSN:0300-8177
1573-4919
DOI:10.1023/A:1006816321679