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Hepatolenticular Degeneration
(Wilson Disease; Pseudosclerosis; Cerebral Pseudosclerosis; Neurohepatic Degeneration)
A rare autosomal recessive disease characterized by the deposition of copper in the brain; LIVER; cornea; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as liver cirrhosis; tremor; ataxia and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
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 disease (hepatolenticular www.medicalassessment.com |  Hepatolenticular Degeneration trialx.com |  Hepatolenticular degeneration eyepathologist.com |  page for hepatolenticular www.textmed.com |
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Wikipedia
Wilson disease protein, Wilson Central High School, Wilson Central School, Wilson's Creek National Battlefield, Wilson College, Mumbai, Woodrow Wilson International Center for Scholars, Thomas Wilson Sons & Co., Wilson, Sompayrac & Urquhart, James A. Wilson, The Wilson Journal of Ornithology
More information
- Reported cases - Summary of cases reported on this disease.
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