Wilson Disease at a Single Cell Level: INTRACELLULAR COPPER TRAFFICKING ACTIVATES COMPARTMENT-SPECIFIC RESPONSES IN HEPATOCYTES

Wilson disease (WD) is a severe hepato-neurologic disorder that affects primarily children and young adults. WD is caused by mutations in ATP7B and subsequent copper overload. However, copper levels alone do not predict severity of the disease. We demonstrate that temporal and spatial distribution o...

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Autori principali: Ralle, Martina, Huster, Dominik, Vogt, Stefan, Schirrmeister, Wiebke, Burkhead, Jason L., Capps, Tony R., Gray, Lawrence, Lai, Barry, Maryon, Edward, Lutsenko, Svetlana
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society for Biochemistry and Molecular Biology 2010
Soggetti:
Molecular Bases of Disease
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2945580/
https://ncbi.nlm.nih.gov/pubmed/20647314
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.114447
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