Article
Wilson disease protein ATP7B utilizes lysosomal exocytosis to maintain copper homeostasis.
Developmental cell - 23 Jun 2014
Polishchuk Elena V, Concilli Mafalda, Iacobacci Simona, Chesi Giancarlo, Pastore Nunzia, Piccolo Pasquale, Paladino Simona, Baldantoni Daniela, van IJzendoorn Sven C D, Chan Jefferson, Chang Christopher J, Amoresano Angela, Pane Francesca, Pucci Piero, Tarallo Antonietta, Parenti Giancarlo, Brunetti-Pierri Nicola, Settembre Carmine, Ballabio Andrea, Polishchuk Roman S
Abstract excerpt
Copper is an essential yet toxic metal and its overload causes Wilson disease, a disorder due to mutations in copper transporter ATP7B. To remove excess copper into the bile, ATP7B traffics toward canalicular area of hepatocytes. However, the trafficking mechanisms of ATP7B remain elusive. Here, we show that, in response to elevated copper, ATP7B moves from the Golgi to lysosomes and imports metal into their...
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