Mitochondrial damage and renewal in Wilson disease will be presented by Prof. Hans Zischka

Prof. Hans Zischka from Institute of Molecular Toxicology and Pharmacology, Germany will speak about "Mitochondrial damage and renewal in Wilson disease".

Wilson disease (WD) is a hereditary defect in the metabolism of copper. Here, the liver is unable to eliminate excess copper causing its accumulation primarily in liver and brain. In neurons and hepatocytes, a rising copper overload particularly damages their mitochondria. However, sometimes years pass before cell death occurs, suggesting the possible existence of intracellular protective mechanisms that can counteract such mitochondrial poisoning.

Prof. Zischka examined cell cultures from WD patients but also WD rodent models for their reaction to copper.

He observed that the hepatocytes reacted to copper overload with an intensification of the mechanisms support autophagy. Particularly, the copper-overloaded mitochondria are affected by this process (in this case called mitophagy). However, mitophagy helps only up to a certain level of copper overload and other treatments need to be employed to avoid cell death. Nevertheless, these results demonstrate the potential for cell regeneration resulting from auto-/mitophagy, which could be also relevant for other disorders that arise or are paralleled by cell-toxic metal accumulations.

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