Function of Copper Transporters

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Presentation transcript:

Function of Copper Transporters By: Stefano Pineda

Overview Copper Importers Ctr1 Ctr2 Copper Exporters ADP7A ADP7B

Distribution of copper

Integral membrane proteins with 3 transmembrane domains that form a homotrimeric pore for import of Cu+ 4 Copper Uptake Ctr1 hCtr1 Integral plasma protein

Where is CTR1 Expressed? Kuo Y et al. PNAS 2001;98:6836-6841

CTR1's Role in Development

Intracellular Copper Distribution

Cu-ATPases Polytopic membrane proteins, transport copper from cytosol to cellular membranes using ATP hydrolysis Transport copper into biosynthetic pathways for subsequent incorporation into cuproenzymes. Delivery to Mitochondria, Secretory pathway, and TGN ATP7A & ATP7B dual function based off intracellular Cu concentration.

Copper Export: Cu-ATPases

Dual Function of Cu-ATPases 1010 Dual Function of Cu-ATPases

ATP7A Copper exported from enterocytes into the blood stream Menkes disease – copper export greatly impaired, copper accumulates in intestine, less copper delivered to blood and tissues. Decreased function of cuproenzymes: connective tissue abnormalities, lack of pigmentation

ATP7B Liver central organ in copper homeostasis Export of excess copper out of the body Hepatic trans-Golgi network contain ATP7B Receive copper from Atox1 transport it into lumen of the TGN Incorporated into copper-dependent ferroxidase ceruloplasmin, most abundant copper containing protein in blood Copper secretion into bile

ATP7A and ATP7B Copper Distribution

In Conclusion CTR1, and Cu-ATPases essential for tight homeostatic copper control Mutations that deviate from WT result in Menke's disease and Wilson's disease Copper transport essential for important physiological processes and development

References Van den Burghe, Peter V. E. and Klomp, Leo W. J. Posttranslational Regulation of Copper Transporters. Journal of Biological Inorganic Chemistry (2010) 15: 37-46 Lutsenko, Svetlana et.al. Function and Regulation of Copper-Transporting ATPases. The American Physiological Society (2007) 87:1011-1046 De Bie, P., Muller, P., et.al. Molecular Pathogenesis of Wilson and Menkes Disease: Correlation of Mutations with Molecular Effects and Disease Phenotypes. Journal of Medical Genetics (2007) 44: 673-688