[Structure and function of ATP7A and ATP7B proteins--Cu-transporting ATPases].
Living organisms have developed refined and geneticaly controlled mechanisms of the copper metabolism and transport. ATP7A and ATP7B proteins play the key role in copper homeostasis in the organism. Both proteins are P-type Cu-transporting ATPases and use the energy of ATP hydrolysis to transfer the copper ions across the cellular membranes. Both proteins are localised in Golgi aparatus and involved in regulation of overall copper status in the body and their function is the export of excess copper from the cells and delivery of copper ions to Cu-dependent enzymes. Moreover in organism Cu-transporting ATPases are involved in absorption of dietary copper, Cu removal with the bile, placental copper transport and its secretion to the milk during lactation. Moreover it is known that Cu-transporting ATPases play a role in generation of anti-cancer drug resistance. Disturbances of ATP7A and ATP7B function caused by mutations lead to severe metabolic diseases Menkes and Wilson diseases, respectively.
Department of Genetics and Evolution, Institute of Zoology, Jagiellonian University, 6 R. Ingardena St., 30-060 Cracow, Poland. malgorzata.lenartowicz@gmail.com
Małgorzata
M
Lenartowicz
Wojciech
W
Krzeptowski
D000251:Adenosine Triphosphatases / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D027682:Cation Transport Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D003300:Copper / Q000378:metabolism*; D000073840:Copper-Transporting ATPases; D019008:Drug Resistance, Neoplasm; D006056:Golgi Apparatus / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000235:genetics; D006706:Homeostasis; D006801:Humans; D007706:Menkes Kinky Hair Syndrome / Q000235:genetics
D027682:Cation Transport Proteins; D003300:Copper; D000251:Adenosine Triphosphatases; C081184:ATP7A protein, human; C000612464:ATP7B protein, human; D000073840:Copper-Transporting ATPases