In a rare inherited disorder, called Wilson’s disease, excessive amounts of copper accumulate in liver and brain tissue. A prominent symptom of the disease is the deposition of copper in greenishbrown layers surrounding the cornea, called Kayser–Fleischer rings. A defective ATP-dependent protein that transports copper across cell membranes causes Wilson’s disease. Apparently, the copper transport protein is required to incorporate copper into ceruloplasmin and to excrete excess copper. In addition to a diet low in copper, Wilson’s disease is treated with zinc sulfate and the chelating agent penicillamine (p. 148). Describe how these treatments work. [Hint: Metallothionein has a greater affinity for copper than for zinc.]

Human Anatomy & Physiology (11th Edition)
11th Edition
ISBN:9780134580999
Author:Elaine N. Marieb, Katja N. Hoehn
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In a rare inherited disorder, called Wilson’s disease, excessive amounts of copper accumulate in
liver and brain tissue. A prominent symptom of the disease is the deposition of copper in greenishbrown
layers surrounding the cornea, called Kayser–Fleischer rings. A defective ATP-dependent
protein that transports copper across cell membranes causes Wilson’s disease. Apparently, the
copper transport protein is required to incorporate copper into ceruloplasmin and to excrete excess
copper. In addition to a diet low in copper, Wilson’s disease is treated with zinc sulfate and the
chelating agent penicillamine (p. 148). Describe how these treatments work. [Hint:
Metallothionein has a greater affinity for copper than for zinc.]

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