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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Main Authors: Ralle, Martina, Huster, Dominik, Vogt, Stefan, Schirrmeister, Wiebke, Burkhead, Jason L., Capps, Tony R., Gray, Lawrence, Lai, Barry, Maryon, Edward, Lutsenko, Svetlana
格式: Artigo
語言:Inglês
出版: American Society for Biochemistry and Molecular Biology 2010
主題:
Molecular Bases of Disease
在線閱讀: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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