Essentials of Biology (5th International Edition)
Essentials of Biology (5th International Edition)
5th Edition
ISBN: 9781259660269
Author: Sylvia S. Mader, Dr., Michael Windelspecht
Publisher: Mcgraw-Hill
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Chapter 5, Problem 2TC

Cystic fibrosis is a genetic disorder that results in a defective membrane transport protein .The defective protein closes chloride ion channels in membranes, preventing chloride ions from being exported out of cells. This results in the development of a thick mucus on the outer surfaces of cells. This mucus clogs the ducts that carry digestive enzymes from the pancreas to the small intestine, clogs the airways in the lungs, and promotes lung infections. Why do you think the defective protein results in a thick, sticky mucus outside the cells, instead of a loose, fluid covering?

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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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