Genetics of Wilson disease*
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Wilson disease is a rare inherited disorder that results in excessive amounts of copper in the body. It is four times more common in females than in males. Copper is normally metabolised by being incorporated into copper-containing enzymes called ceruloplasmin and being excreted into the bile. However, in Wilson disease, the process is impaired and excess copper is initially deposited in the liver where it damages liver cells. Eventually, as liver copper levels increase, it is released into the blood and deposited in other organs, particularly the brain and spinal cord. The copper deposits cause tissue damage, tissue death, and scarring, which cause the affected organs to stop functioning properly. Wilson disease is also known as hepatolenticular degeneration.
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