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Copper Ligands in Human Milk: A Vehicle for Copper Supplementation in the Treatment of Menkes' Disease?

Copper Ligands in Human Milk: A Vehicle for Copper Supplementation in the Treatment of Menkes'... Abstract To the Editor.—Recent results from our laboratory have shown that human milk, unlike cow's milk, contains a low-molecular-weight (LMW) zinc complex,1 now identified as zinc citrate.2 We have therefore hypothesized that the therapeutic value of human milk in the treatment of acrodermatitis enteropathica (AE), a genetic disorder of zinc metabolism, is due to the presence of this complex, which is thought to be highly available for absorption.3,4 Assuming that human milk may contain LMW copper as well as zinc complexes, Williams et al5 have suggested that human milk may also be of therapeutic value in the treatment of Menkes' kinky hair disease, a sexlinked genetic disorder in humans that is manifested by abnormal intestinal copper absorption and many characteristics similar to those of copper-deficient animals.6 Despite the aggressive use of copper in infants with Menkes' disease, the prognosis for these patients has not improved. References 1. Eckhert CD, Sloan M, Duncan J, et al: Zinc binding: A difference between human and bovine milk . Science 195:789-790, 1977.Crossref 2. Lönnerdal B, Stanislowski AG, Hurley LS: Isolation of a low molecular weight zinc binding ligand from human milk . J Inorg Biochem 12:71-78, 1980.Crossref 3. Giroux E, Prakash NJ: Influence of zincligand mixtures on serum zinc levels in rats . J Pharm Sci 66:391-395, 1977.Crossref 4. Hurley LS, Lönnerdal B, Stanislowski AG: Zinc citrate, human milk and acrodermatitis enteropathica . Lancet 1:677-678, 1979.Crossref 5. Williams DM, Atkin CL, Seay AR, et al: Failure of human milk therapy in Menkes' kinky hair disease . Am J Dis Child 133:218-219, 1979. 6. Hurley LS, Keen CL: Teratogenic effects of copper , in Niagru J (ed): Copper in the Environment , (part 2) , Health Effects. New York, John Wiley & Sons Inc, 1979, pp 33-56. 7. Garnica AD, Frias JL, Rennert OM: Menkes' kinky hair syndrome: Is it a treatable disorder? Clin Gen 11:154-161, 1977.Crossref 8. Danks DM, Camakaris J, Stevens BJ: The cellular defect in Menkes' syndrome and in mottled mice , in Kirchgessner M (ed): Trace Element Metabolism in Man and Animals . Technische Universität München, Friesing-Weihenstephan, 1978, vol 3, pp 401-404. 9. Lönnerdal B: Chemical modification of dextran gels for gel filtration of trace element ligands. Read before the First International Workshop on Trace Element Analytical Chemistry in Medicine Biology, Neuherberg, Federal Republic of Germany, April 27-29, 1980. 10. Fransson G-B, Lönnerdal B: Iron in human milk . J Pediatr 96:380-384, 1980.Crossref 11. Hurley LS, Bell LT: Amelioration by copper supplementation of mutant gene effects in the crinkled mouse . Proc Soc Exp Biol Med 149:830-834, 1975. 12. Keen CL, Saltman P, Hurley LS: Copper nitrilotriacetate: A potent therapeutic agent in the treatment of a genetic disorder of copper metabolism. Am J Clin Nutr, to be published. 13. Erway L, Hurley LS, Fraser A: Neurological defect: Manganese in phenocopy and prevention of a genetic abnormality of inner ear . Science 152:1766-1768, 1966.Crossref 14. Erway LC, Mitchell SE: Prevention of otolith defect in pastel mink by manganese supplementation . J Hered 64:110-119, 1973. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Copper Ligands in Human Milk: A Vehicle for Copper Supplementation in the Treatment of Menkes' Disease?

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Publisher
American Medical Association
Copyright
Copyright © 1980 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1980.02130200070029
Publisher site
See Article on Publisher Site

Abstract

Abstract To the Editor.—Recent results from our laboratory have shown that human milk, unlike cow's milk, contains a low-molecular-weight (LMW) zinc complex,1 now identified as zinc citrate.2 We have therefore hypothesized that the therapeutic value of human milk in the treatment of acrodermatitis enteropathica (AE), a genetic disorder of zinc metabolism, is due to the presence of this complex, which is thought to be highly available for absorption.3,4 Assuming that human milk may contain LMW copper as well as zinc complexes, Williams et al5 have suggested that human milk may also be of therapeutic value in the treatment of Menkes' kinky hair disease, a sexlinked genetic disorder in humans that is manifested by abnormal intestinal copper absorption and many characteristics similar to those of copper-deficient animals.6 Despite the aggressive use of copper in infants with Menkes' disease, the prognosis for these patients has not improved. References 1. Eckhert CD, Sloan M, Duncan J, et al: Zinc binding: A difference between human and bovine milk . Science 195:789-790, 1977.Crossref 2. Lönnerdal B, Stanislowski AG, Hurley LS: Isolation of a low molecular weight zinc binding ligand from human milk . J Inorg Biochem 12:71-78, 1980.Crossref 3. Giroux E, Prakash NJ: Influence of zincligand mixtures on serum zinc levels in rats . J Pharm Sci 66:391-395, 1977.Crossref 4. Hurley LS, Lönnerdal B, Stanislowski AG: Zinc citrate, human milk and acrodermatitis enteropathica . Lancet 1:677-678, 1979.Crossref 5. Williams DM, Atkin CL, Seay AR, et al: Failure of human milk therapy in Menkes' kinky hair disease . Am J Dis Child 133:218-219, 1979. 6. Hurley LS, Keen CL: Teratogenic effects of copper , in Niagru J (ed): Copper in the Environment , (part 2) , Health Effects. New York, John Wiley & Sons Inc, 1979, pp 33-56. 7. Garnica AD, Frias JL, Rennert OM: Menkes' kinky hair syndrome: Is it a treatable disorder? Clin Gen 11:154-161, 1977.Crossref 8. Danks DM, Camakaris J, Stevens BJ: The cellular defect in Menkes' syndrome and in mottled mice , in Kirchgessner M (ed): Trace Element Metabolism in Man and Animals . Technische Universität München, Friesing-Weihenstephan, 1978, vol 3, pp 401-404. 9. Lönnerdal B: Chemical modification of dextran gels for gel filtration of trace element ligands. Read before the First International Workshop on Trace Element Analytical Chemistry in Medicine Biology, Neuherberg, Federal Republic of Germany, April 27-29, 1980. 10. Fransson G-B, Lönnerdal B: Iron in human milk . J Pediatr 96:380-384, 1980.Crossref 11. Hurley LS, Bell LT: Amelioration by copper supplementation of mutant gene effects in the crinkled mouse . Proc Soc Exp Biol Med 149:830-834, 1975. 12. Keen CL, Saltman P, Hurley LS: Copper nitrilotriacetate: A potent therapeutic agent in the treatment of a genetic disorder of copper metabolism. Am J Clin Nutr, to be published. 13. Erway L, Hurley LS, Fraser A: Neurological defect: Manganese in phenocopy and prevention of a genetic abnormality of inner ear . Science 152:1766-1768, 1966.Crossref 14. Erway LC, Mitchell SE: Prevention of otolith defect in pastel mink by manganese supplementation . J Hered 64:110-119, 1973.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Aug 1, 1980

References