Article
A systems biology approach reveals new endoplasmic reticulum-associated targets for the correction of the ATP7B mutant causing Wilson disease.
Metallomics : integrated biometal science - 1 Sept 2016
Concilli Mafalda, Iacobacci Simona, Chesi Giancarlo, Carissimo Annamaria, Polishchuk Roman
Abstract excerpt
Copper (Cu) is an important trace element required for the activity of essential enzymes. However, excess Cu compromises the redox balance in cells and tissues causing serious toxicity. The process of disposal of excess Cu from organisms relies on the activity of Cu-transporting ATPase ATP7B. ATP7B is mainly expressed in liver hepatocytes where it sequesters the potentially toxic metal and mediates its excretion...
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