Published in Am J Clin Nutr on May 01, 1996
Interaction of the copper chaperone HAH1 with the Wilson disease protein is essential for copper homeostasis. Proc Natl Acad Sci U S A (1999) 1.83
Molecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypes. J Med Genet (2007) 1.66
Physiological and toxicological transcriptome changes in HepG2 cells exposed to copper. Physiol Genomics (2009) 0.96
Gene expression profiling of liver cells after copper overload in vivo and in vitro reveals new copper-regulated genes. J Biol Inorg Chem (2007) 0.96
Animal models of copper-associated liver disease. Comp Hepatol (2003) 0.86
Copper activation of NF-kappaB signaling in HepG2 cells. J Mol Biol (2009) 0.85
Canine models of copper toxicosis for understanding mammalian copper metabolism. Mamm Genome (2011) 0.79
Liver transplantation for Wilson disease. World J Hepatol (2012) 0.79
Optical coherence tomography and electrophysiology of retinal and visual pathways in Wilson's disease. Metab Brain Dis (2015) 0.78
Administration of PPARβ/δ agonist reduces copper-induced liver damage in mice: possible implications in clinical practice. J Clin Biochem Nutr (2011) 0.77
High frequency of the c.3207C>A (p.H1069Q) mutation in ATP7B gene of Lithuanian patients with hepatic presentation of Wilson's disease. World J Gastroenterol (2008) 0.77
A Patient With Primary Biliary Cirrhosis Accompanied by Wilson's Disease. Hepat Mon (2016) 0.75
The effect of dietary zinc--and polyphenols intake on DMBA-induced mammary tumorigenesis in rats. J Biomed Sci (2012) 0.75
Canine Models for Copper Homeostasis Disorders. Int J Mol Sci (2016) 0.75
D-penicillamine induced Goodpasture's syndrome in Wilson's disease. Ann Intern Med (1975) 2.56
Prevention of Wilson's disease in asymptomatic patients. N Engl J Med (1968) 2.11
Physical and chemical studies on ceruloplasmin. V. Metabolic studies on sialic acid-free ceruloplasmin in vivo. J Biol Chem (1968) 1.80
Identification and analysis of mutations in the Wilson disease gene (ATP7B): population frequencies, genotype-phenotype correlation, and functional analyses. Am J Hum Genet (1997) 1.79
Decreased taste sensitivity after D-penicillamine reversed by copper administration. Lancet (1967) 1.69
Chronic hepatitis as a first manifestation of Wilson's disease. Ann Intern Med (1972) 1.64
Orthotopic liver transplantation for Wilson's disease. Lancet (1971) 1.60
The peroxisomes of human hepatocytes. Lab Invest (1977) 1.50
Copper and the liver. Gastroenterology (1980) 1.50
Catabolism of desialylated ceruloplasmin in the liver. J Biol Chem (1970) 1.47
Is non-Indian childhood cirrhosis caused by excess dietary copper? Lancet (1994) 1.43
Endocytosis of asialoglycoprotein-enzyme conjugates by hepatocytes. Lab Invest (1980) 1.42
The role of radiocopper in the diagnosis of Wilson's disease. Gastroenterology (1979) 1.37
Changes in the distribution of hepatic copper in relation to the progression of Wilson's disease (hepatolenticular degeneration). Am J Pathol (1968) 1.33
Evolution of the hepatic lesion in Wilson's disease (hepatolenticular degeneration). Prog Liver Dis (1972) 1.30
IgA interaction with the asialoglycoprotein receptor. Proc Natl Acad Sci U S A (1982) 1.29
Elastosis perforans serpiginosa during penicillamine therapy for Wilson disease. Arch Dermatol (1973) 1.26
Liver transplantation for Wilson's disease: indications and outcome. Hepatology (1994) 1.24
The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson's disease. N Engl J Med (1987) 1.23
Skeletal changes in Wilson's disease. A radiological study. Radiology (1970) 1.20
Measurement of gastrointestinal protein loss using ceruloplasmin labeled with copper. J Clin Invest (1967) 1.19
The significance of variations in the distribution of copper in liver disease. Am J Pathol (1980) 1.17
Clinical, morphologic, and chemical studies on copper toxicosis of Bedlington Terriers. J Am Vet Med Assoc (1979) 1.16
Cytoplasmic copper and its toxic effects. Studies in Indian childhood cirrhosis. Lancet (1979) 1.16
Orthotopic liver transplantation for Wilson's disease: a single-center experience. Transplantation (2001) 1.08
Lysosomal defect of hepatic copper excretion in Wilson's disease (hepatolenticular degeneration). Gastroenterology (1973) 1.08
Eight closely linked loci place the Wilson disease locus within 13q14-q21. Am J Hum Genet (1988) 1.07
Mitochondrial and fatty changes in hepatocytes of patients with Wilson's disease. Gastroenterology (1968) 1.07
Diagnosis of Wilson's disease. Gastroenterology (1978) 1.06
Morphologic and chemical studies on a murine mutation (toxic milk mice) resulting in hepatic copper toxicosis. Lab Invest (1988) 1.06
Predictive testing for Wilson's disease using tightly linked and flanking DNA markers. Neurology (1991) 1.01
Optical diffraction studies of crystalline structures in electron micrographs. II. Crystalline inclusions in mitochondria of human hepatocytes. J Cell Biol (1969) 1.00
Pregnancy in penicillamine-treated patients with Wilson's disease. N Engl J Med (1975) 0.99
Wilson's disease: indications for liver transplants. Hepatology (1984) 0.98
Overcoming obstacles to the diagnosis of Wilson's disease. Gastroenterology (1997) 0.98
Hepatic lysosomal copper protein in dogs with an inherited copper toxicosis. Hepatology (1981) 0.96
Hepatic peroxisomal abnormalities in abetalipoproteinemia. Gastroenterology (1989) 0.94
Molecular studies of ceruloplasmin deficiency in Wilson's disease. J Clin Invest (1987) 0.94
Special article: functional implications of human portal and bile ductular ultrastructure. Gastroenterology (1972) 0.91
Inheritance of copper toxicosis in Bedlington terriers. Am J Vet Res (1980) 0.90
[Copper in biliary cirrhosis in children]. Rev Int Hepatol (1966) 0.90
Copper metallothionein in patients with hepatic copper overload. Eur J Clin Invest (1991) 0.89
The cytopathology of metal overload. Int Rev Exp Pathol (1990) 0.89
Hepatocellular copper toxicity and its attenuation by zinc. J Clin Invest (1989) 0.89
Detection of multiple forms of human ceruloplasmin. A novel Mr 200,000 form. J Biol Chem (1990) 0.88
Spontaneous cholangiofibrosis in Long-Evans Cinnamon rats: a rodent model for Wilson's disease. Lab Anim Sci (1998) 0.88
Systemic absorption of intrauterine copper. Science (1972) 0.88
Dosimetry of copper radionuclides. J Nucl Med (1974) 0.87
Effects of anticopper therapy on hepatocellular mitochondria in patients with Wilson's disease: an ultrastructural and stereological study. Gastroenterology (1976) 0.87
Chronic active hepatitis in Doberman pinschers. J Am Vet Med Assoc (1982) 0.86
Nonceruloplasmin copper in rheumatoid arthritis. Arthritis Rheum (1969) 0.86
Penicillamine and the nephrotic syndrome. JAMA (1966) 0.85
Cytoplasmic crystals in human hepatocytes. Lab Invest (1971) 0.85
Penicillamine may detoxify copper in Wilson's disease. Lancet (1987) 0.85
A prospective clinical trial of D-penicillamine in the treatment of primary biliary cirrhosis. Hepatology (1985) 0.85
Heavy metals and lysosomes. Front Biol (1976) 0.84
Lipolysosomes in human hepatocytes. Ultrastructural and cytochemical studies of patients with Wilson's disease. Lab Invest (1975) 0.84
Pleiotropic effect of LEC mutation: a rodent model of Wilson's disease. Am J Physiol (1994) 0.83
Biliary proteins and ductular ultrastructure. Hepatology (1985) 0.82
Transport and intracellular distribution of copper in a human hepatoblastoma cell line, HepG2. Hepatology (1986) 0.82
Canine hepatic lysosomal copper protein: identification as metallothionein. Arch Biochem Biophys (1985) 0.82
Defective urinary acidification in Wilson's disease. Ann Intern Med (1968) 0.82
Penicillamine-induced skin lesions. J Rheumatol Suppl (1981) 0.82
Bleeding oesophageal varices in patients with Wilson's disease. Lancet (1970) 0.81
Copper in hepatocyte lysosomes of the toad, Bufo marinus L. Nature (1970) 0.81
The application of laser microprobe mass analysis to the study of biological material. Biometals (1996) 0.80
The development of cirrhosis in Wilson's disease. Clin Gastroenterol (1975) 0.80
R-bodies of human rectal epithelial cells. Arch Pathol Lab Med (1976) 0.80
Neuropathological findings in penicillamine-treated patients with Wilson's disease. Clin Neuropathol (1988) 0.79
Clinical aspects of Wilson's disease. Am J Gastroenterol (1990) 0.79
Prognosis of Wilsonian chronic active hepatitis. Gastroenterology (1991) 0.79
Radiocopper in diagnosing liver disease. Semin Nucl Med (1972) 0.78
Mitochondrion-desmosome complexes in human hepatocytes. Z Zellforsch Mikrosk Anat (1969) 0.78
Electron microscopy of mitochondria and peroxisomes of human hepatocytes. Prog Liver Dis (1979) 0.78
Wilson's disease and Kayser-Fleischer rings. Ann Neurol (1986) 0.78
Wilson's disease and pregnancy. Hepatology (2000) 0.78
The cardiomyopathy of Wilson's disease. Myocardial alterations in nine cases. Virchows Arch A Pathol Anat Histol (1982) 0.78
Prophylaxis of Wilson's disease. N Engl J Med (1968) 0.77
Study of a possible physiologic interaction between ceruloplasmin and serotonin. J Lab Clin Med (1966) 0.77
An international symposium on Wilson's and Menkes' diseases. Hepatology (1996) 0.77
Cytochemical detection of inherited copper toxicosis of Bedlington terriers. Vet Pathol (1984) 0.76
Copper toxicosis of the Bedlington terrier. J Rheumatol Suppl (1981) 0.76
Salicylazosulfapyridine in inflammatory bowel disease. Am J Dig Dis (1975) 0.76
Marginal plate in human hepatic microbody. N Engl J Med (1970) 0.76
Origins of biliary copper. Hepatology (1984) 0.75
Light- and electron-microscopical study of a case of gold salt-induced hepatotoxicity. Hepatology (1991) 0.75
Excess zinc associated with cholestasis. Lancet (1996) 0.75
Treatment of the neurologic manifestations of Wilson's disease. Arch Neurol (1995) 0.75
Familial cholestatic cirrhosis associated with Kayser-Fleischer rings. Pediatrics (1980) 0.75
Ribosome orientation in Mallory bodies. Gastroenterology (1987) 0.75
The remarkable selectivity of hepatocytes in the uptake of glycoproteins. Gastroenterology (1973) 0.75
[Wilson's disease (hepatocerebral degeneration)]. Internist (Berl) (1976) 0.75
Physical and chemical studies on ceruloplasmin: crystallization of desialized human ceruloplasmin asialoceruloplasmin. Science (1969) 0.75
The effects of two new inotropic agents on microsomal liver function in patients with congestive heart failure. Am J Med Sci (1986) 0.75
[Recent acquisitions in the study of Wilson's disease]. Rev Med Chir Mal Foie (1965) 0.75
[Diagnosis of Wilson's disease in 1981]. Gastroenterol Clin Biol (1981) 0.75
Letter: Diagnosis of Wilson's disease. Gastroenterology (1974) 0.75
[D-penicillamine]. Med Chir Dig (1973) 0.75
Lipolysosomes in human liver: distribution in livers with fatty infiltration. Gastroenterology (1977) 0.75