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 of copper in hepatocytes may play an important role in WD
pathology. High resolution synchrotron-based x-ray fluorescence imaging in situ indicates
that copper does not continuously accumulate in Atp7b−/− hepatocytes, but reaches a limit …
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 of copper in hepatocytes may play an important role in WD
pathology. High resolution synchrotron-based x-ray fluorescence imaging in situ indicates
that copper does not continuously accumulate in Atp7b−/− hepatocytes, but reaches a limit …
