Copper is a cofactor in several enzymes, including lysyl oxidase and superoxide dismutase.Ceruloplasmin, a deep-blue glycoprotein, is the principal copper-containing protein in blood. It isused to transport Cu2+ and maintain appropriate levels of Cu2+ in the body’s tissues.Ceruloplasmin also catalyzes the oxidation of Fe2+ to Fe3+, an important reaction in ironmetabolism. Because the metal is widely found in foods, copper deficiency is rare in humans.Deficiency symptoms include anemia, leukopenia (reduction in blood levels of white blood cells),bone defects, and weakened arterial walls. The body is partially protected from exposure toexcessive copper (and several other metals) by metallothionein, a small, metal-binding protein thatpossesses a large proportion of cysteine residues. Certain metals (most notably zinc and cadmium)induce the synthesis of metallothionein in the intestine and liver.In Menkes syndrome intestinal absorption of copper is defective. How can affected infants betreated to avoid the symptoms of the disorder, which include seizures, retarded growth, and brittlehair?

Human Anatomy & Physiology (11th Edition)
11th Edition
ISBN:9780134580999
Author:Elaine N. Marieb, Katja N. Hoehn
Publisher:Elaine N. Marieb, Katja N. Hoehn
Chapter1: The Human Body: An Orientation
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Copper is a cofactor in several enzymes, including lysyl oxidase and superoxide dismutase.
Ceruloplasmin, a deep-blue glycoprotein, is the principal copper-containing protein in blood. It is
used to transport Cu2+ and maintain appropriate levels of Cu2+ in the body’s tissues.
Ceruloplasmin also catalyzes the oxidation of Fe2+ to Fe3+, an important reaction in iron
metabolism. Because the metal is widely found in foods, copper deficiency is rare in humans.
Deficiency symptoms include anemia, leukopenia (reduction in blood levels of white blood cells),
bone defects, and weakened arterial walls. The body is partially protected from exposure to
excessive copper (and several other metals) by metallothionein, a small, metal-binding protein that
possesses a large proportion of cysteine residues. Certain metals (most notably zinc and cadmium)
induce the synthesis of metallothionein in the intestine and liver.
In Menkes syndrome intestinal absorption of copper is defective. How can affected infants be
treated to avoid the symptoms of the disorder, which include seizures, retarded growth, and brittle
hair?

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