AJCN EB Program 2010 Early Registration
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Purchase Article
Right arrow View Shopping Cart
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Scheinberg, I. H.
Right arrow Articles by Sternlieb, I.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Scheinberg, I. H.
Right arrow Articles by Sternlieb, I.
Agricola
Right arrow Articles by Scheinberg, I. H.
Right arrow Articles by Sternlieb, I.

American Journal of Clinical Nutrition, Vol 63, 842S-845S, Copyright © 1996 by The American Society for Clinical Nutrition, Inc

REVIEW ARTICLES

Wilson disease and idiopathic copper toxicosis

IH Scheinberg and I Sternlieb
National Center for the Study of Wilson's Disease, St Luke's-Roosevelt Hospital Center, Columbia University, New York, USA.

The pathogenic agent of both Wilson disease (WD) and non-Indian childhood cirrhosis (which we term idiopathic copper toxicosis, or ICT) is copper accumulating to excess in the liver. Inheritance of a pair of alleles of an autosomal recessive gene on chromosome 13 is necessary and sufficient to cause such copper accumulation in WD; reducing the dietary intake of copper cannot prevent the development of WD. In contrast, the lethal accumulations of copper in children with ICT have been attributed primarily to an increased dietary intake of copper. However, 64 124 child-year exposures of children under the age of 6 y to drinking water containing a copper concentration of approximately 125.9 micromol/L (8 mg/L) produced no deaths from any form of liver disease. Moreover, the ICT of seven infants was attributed primarily to drinking water containing < 110.2 micromol Cu/L (7 mg/L) despite evidence of the presence of a genetic defect in three of the patients, one of whom was exclusively breast-fed. These data suggest that ICT cannot be caused solely by increased dietary intake of copper and occurs only in children with an identified genetic defect.


This article has been cited by other articles:


Home page
 Am. J. Physiol. Gastrointest. Liver Physiol.Home page
K. A. Bauerly, S. L. Kelleher, and B. Lonnerdal
Effects of copper supplementation on copper absorption, tissue distribution, and copper transporter expression in an infant rat model
Am J Physiol Gastrointest Liver Physiol, May 1, 2005; 288(5): G1007 - G1014.
[Abstract] [Full Text] [PDF]

Home page
 Annals of Clinical & Laboratory ScienceHome page
K. L. Nuttall, J. Palaty, and G. Lockitch
Reference Limits for Copper and Iron in Liver Biopsies
Ann. Clin. Lab. Sci., October 1, 2003; 33(4): 443 - 450.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Regul. Integr. Comp. Physiol.Home page
M. Grosell, J. C. McGeer, and C. M. Wood
Plasma copper clearance and biliary copper excretion are stimulated in copper-acclimated trout
Am J Physiol Regulatory Integrative Comp Physiol, March 1, 2001; 280(3): R796 - R806.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
I. Hamza, M. Schaefer, L. W. J. Klomp, and J. D. Gitlin
Interaction of the copper chaperone HAH1 with the Wilson disease protein is essential for copper homeostasis
PNAS, November 9, 1999; 96(23): 13363 - 13368.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Gastrointest. Liver Physiol.Home page
M. Schaefer, R. G. Hopkins, M. L. Failla, and J. D. Gitlin
Hepatocyte-specific localization and copper-dependent trafficking of the Wilson's disease protein in the liver
Am J Physiol Gastrointest Liver Physiol, March 1, 1999; 276(3): G639 - G646.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 1996 by The American Society for Nutrition