1. Wilson Disease Mayo Clinic Proceedings
Mounif El-Youssef, MD 
Mayo Clinic Proceedings 
Volume 78, Issue 9, Pages (September 2003)
DOI: /
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2. Figure 1
Pathway of copper metabolism. The bar represents the metabolic abnormality of Wilson disease. In the absence of normal ATP7B, intracellular trafficking of copper is inadequate, resulting in copper accumulation and increased degradation of the apoceruloplasmin form of the protein and subsequent low serum level. Excess copper eventually saturates the liver and other organs.
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3. Figure 2
Schematic representation of the transport mechanism of copper in the hepatocyte. The metal is transported into the canalicular membrane with the help of the ATP7B molecule from the trans-Golgi. Copper in the cytosol is incorporated into apo-ceruloplasmin for transportation to various enzymes and for plasma secretion. In the absence of normal transport, the copper is not excreted into bile, and ceruloplasmin is rapidly metabolized, leading to low serum levels and copper toxicosis.
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4. Figure 3
Distribution of hepatic and neurologic manifestations of Wilson disease by age. Note that hepatic manifestations predominate in the pediatric age group in contrast to the neurologic manifestations in individuals older than 18 years.
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5. Figure 4 Distribution of hepatic presentations of Wilson disease.
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6. Figure 5
Features typical of chronic hepatitis with portal inflammation by lymphocytic infiltration and piecemeal necrosis spilling into the parenchyma beyond the limiting plate. There is evidence of lobular disarray with hepatocyte cords that seem to be disrupted.
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7. Figure 6
Steatosis and steatohepatitis associated with the accumulation of fat in a patient with Wilson disease. It is presumed that mitochondrial injury causes disruption of fatty acid oxidation with resultant lipid accumulation inside the hepatocytes.
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8. Figure 7
Established cirrhosis in a patient with Wilson disease. Note the extensive fibrosis bridging from one portal area to the next.
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9. Figure 8
P-type adenosine triphosphatase (ATPase) is responsible for the binding and transport of copper. Note the 6 copper-binding domains, the adenosine triphosphate (ATP)–binding domain, the phosphorylation domain, and the channel domain.
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