The treatment that can be administered to infants suffering from Menkes’ syndrome, so that the symptoms of the disorder can be avoided. Introduction: Copper acts as a cofactor for enzymes like lysyl oxidase and superoxide dismutase. Copper is mainly present in the protein called ceruloplasmin, which is a deep blue glycoprotein. Itsmajor function is to transport and maintain the levels of the copper (Cu 2+ ) ions in the body. Ceruloplasmin also maintains the iron metabolism .

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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 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…

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Chapter 6, Problem 6Q

Summary Introduction

To review:

The treatment that can be administered to infants suffering from Menkes’ syndrome, so that the symptoms of the disorder can be avoided.

Introduction:

Copper acts as a cofactor for enzymes like lysyl oxidase and superoxide dismutase. Copper is mainly present in the protein called ceruloplasmin, which is a deep blue glycoprotein. Itsmajor function is to transport and maintain the levels of the copper (Cu²⁺ ) ions in the body. Ceruloplasmin also maintains the iron metabolism.

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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 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…

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