PMID: 8615372May 1, 1996Paper

Wilson disease and idiopathic copper toxicosis

The American Journal of Clinical Nutrition
I H Scheinberg, I Sternlieb

Abstract

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.

Citations

Nov 11, 1999·Proceedings of the National Academy of Sciences of the United States of America·I HamzaJ D Gitlin
Oct 27, 1999·Biological Trace Element Research·C W LevensonE C Yeiser
Feb 15, 2001·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·M GrosellC M Wood
Dec 11, 2002·Journal of Gastroenterology and Hepatology·Anand N Pankit, Sheila A Bhave
Apr 2, 2004·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Thomas MüllerHelmut Denk
Oct 30, 2004·Biotechnology and Bioengineering·Ranjit S ShettySylvia Daunert
Nov 24, 2004·The Veterinary Quarterly·P J J MandigersJ Rothuizen
Apr 24, 2008·Toxicology and Industrial Health·Selene ChouChristopher De Rosa
Jan 28, 2009·Journal of Pediatric Gastroenterology and Nutrition·Nina ManolakiVassiliki P Syriopoulou
Jan 16, 2010·Journal of Toxicology and Environmental Health. Part a·Bonnie Ransom Stern
Jul 19, 2011·Journal of Clinical Biochemistry and Nutrition·Alvaro A Sanchez-SilesYoshikazu Kinoshita
Dec 8, 2011·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Hille FietenJan Rothuizen
May 7, 2014·Movement Disorders : Official Journal of the Movement Disorder Society·Aggarwal Annu, Bhatt Mohit
Sep 1, 2017·World Journal of Pediatrics : WJP·Hong-Wen ZhuYu-Min Li
Apr 1, 2017·Nutrition in Clinical Practice : Official Publication of the American Society for Parenteral and Enteral Nutrition·Callum Livingstone
Feb 24, 2001·Journal of Toxicology. Clinical Toxicology·I C FuentealbaG M Cherian
Jun 26, 1999·Journal of Toxicology. Clinical Toxicology·D G Barceloux
Jun 25, 2009·Physiological Genomics·Min Ok SongJonathan H Freedman
Sep 13, 2007·Clinical Toxicology : the Official Journal of the American Academy of Clinical Toxicology and European Association of Poisons Centres and Clinical Toxicologists·Teresa LechBogdan Kosowski
Jan 11, 2007·Journal of Biological Inorganic Chemistry : JBIC : a Publication of the Society of Biological Inorganic Chemistry·Patricia MullerLeo W J Klomp
Mar 5, 2011·Journal of Fluorescence·Mansoor Ahmed QaziShahabuddin Memon
Sep 25, 2012·Journal of Veterinary Internal Medicine·H FietenJ Rothuizen
Jan 14, 2016·Molecular Neurobiology·Rosanna SquittiCristian Bonvicini
May 6, 2016·Hepatitis Monthly·Su-Xian ZhaoYue-Min Nan
Jun 5, 2019·Medical Molecular Morphology·Masaru HaradaMichihiko Shibata
Aug 15, 2000·Journal of Pediatric Gastroenterology and Nutrition·M OlivaresR Uauy
Dec 1, 2006·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Daphne M Y CheahKatrina J Allen
Apr 25, 2007·Journal of Toxicology and Environmental Health. Part B, Critical Reviews·Bonnie Ransom SternTom Starr
Sep 30, 1999·Human Mutation·D CurtisS Tanner
Sep 4, 2012·Indian Journal of Gastroenterology : Official Journal of the Indian Society of Gastroenterology·S Suresh KumarEve A Roberts
Feb 9, 2012·World Journal of Hepatology·Andreea M Catana, Valentina Medici
Nov 21, 2012·Chemical Biology & Drug Design·Xuan Tang, Xiangyang Liang
Dec 22, 2015·Metabolic Brain Disease·Ewa Langwińska-WośkoAnna Członkowska
Sep 2, 2016·Journal of Biosciences·S R Bharathi DeviK N Sulochana
Jul 19, 2017·Liver International : Official Journal of the International Association for the Study of the Liver·Olivier GuillaudMuriel Bost
Apr 10, 2014·Movement Disorders Clinical Practice·Annu Aggarwal, Mohit Bhatt
Apr 2, 2020·International Journal of Molecular Sciences·Barbara WittTanja Schwerdtle
Sep 10, 2020·Frontiers in Bioengineering and Biotechnology·Iris RibitschLouis C Penning
Feb 14, 2007·Journal of Gastroenterology and Hepatology·Akiko SaitoHiroshi Hara
Jun 29, 2011·Biological Trace Element Research·Berrin Kocaoğlu GüçlüMurat Kanbur

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Clinics and Research in Hepatology and Gastroenterology
Fabienne DufernezDominique Debray
European Journal of Medical Research
N I Walker
© 2021 Meta ULC. All rights reserved