Ontology highlight
ABSTRACT:
SUBMITTER: Polishchuk EV
PROVIDER: S-EPMC4070386 | biostudies-literature | 2014 Jun
REPOSITORIES: biostudies-literature
Polishchuk Elena V EV Concilli Mafalda M Iacobacci Simona S Chesi Giancarlo G Pastore Nunzia N Piccolo Pasquale P Paladino Simona S Baldantoni Daniela D van IJzendoorn Sven C D SC Chan Jefferson J Chang Christopher J CJ Amoresano Angela A Pane Francesca F Pucci Piero P Tarallo Antonietta A Parenti Giancarlo G Brunetti-Pierri Nicola N Settembre Carmine C Ballabio Andrea A Polishchuk Roman S RS
Developmental cell 20140605 6
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 lumen. ATP7B enables lysosomes to undergo exocytosis through the interaction with p62 su ...[more]