Published in Lab Invest on October 01, 1988
In vivo reduction of amyloid-beta by a mutant copper transporter. Proc Natl Acad Sci U S A (2003) 1.52
Consequences of copper accumulation in the livers of the Atp7b-/- (Wilson disease gene) knockout mice. Am J Pathol (2006) 1.47
Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease. J Clin Invest (1991) 1.10
Prospective evaluation of the diagnostic accuracy of hepatic copper content, as determined using the entire core of a liver biopsy sample. Hepatology (2015) 0.98
Systems biology approach to Wilson's disease. Biometals (2011) 0.94
A mutant mouse (tx) with increased hepatic metallothionein stability and accumulation. Biochem J (1993) 0.91
Maternal choline modifies fetal liver copper, gene expression, DNA methylation, and neonatal growth in the tx-j mouse model of Wilson disease. Epigenetics (2013) 0.91
Wilson's disease: changes in methionine metabolism and inflammation affect global DNA methylation in early liver disease. Hepatology (2013) 0.89
Hepatocellular copper toxicity and its attenuation by zinc. J Clin Invest (1989) 0.89
Animal models of copper-associated liver disease. Comp Hepatol (2003) 0.86
Thiamine supplementation attenuated hepatocellular carcinoma in the Atp7b mouse model of Wilson's disease. Anticancer Res (2011) 0.83
Characterization of timed changes in hepatic copper concentrations, methionine metabolism, gene expression, and global DNA methylation in the Jackson toxic milk mouse model of Wilson disease. Int J Mol Sci (2014) 0.78
Early stage transplantation of bone marrow cells markedly ameliorates copper metabolism and restores liver function in a mouse model of Wilson disease. BMC Gastroenterol (2011) 0.77
Simultaneous monitoring of cerebral metal accumulation in an experimental model of Wilson's disease by laser ablation inductively coupled plasma mass spectrometry. BMC Neurosci (2014) 0.76
A proteomics study of the response of North Ronaldsay sheep to copper challenge. BMC Vet Res (2006) 0.75
Decreased carbonic anhydrase III levels in the liver of the mouse mutant 'toxic milk' (tx) due to copper accumulation. Biochem J (1997) 0.75
Golgi apparatus, GERL, and lysosomes of neurons in rat dorsal root ganglia, studied by thick section and thin section cytochemistry. J Cell Biol (1971) 5.94
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
Studies of the secretory process in the mammalian exocrine pancreas. I. The condensing vacuoles. J Cell Biol (1977) 1.97
Studies on microperoxisomes. II. A cytochemical method for light and electron microscopy. J Histochem Cytochem (1972) 1.82
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
Diffusion artificats in 3,3'-diaminobenzidine cytochemistry. J Histochem Cytochem (1972) 1.47
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
Toxic milk, a new mutation affecting cooper metabolism in the mouse. J Hered (1983) 1.37
The role of radiocopper in the diagnosis of Wilson's disease. Gastroenterology (1979) 1.37
Nucleoside phosphatase and cholinesterase activities in dorsal root ganglia and peripheral nerve. J Cell Biol (1966) 1.34
Changes in the distribution of hepatic copper in relation to the progression of Wilson's disease (hepatolenticular degeneration). Am J Pathol (1968) 1.33
Studies on microperoxisomes. V. Are microperoxisomes ubiquitous in mammalian cells? J Histochem Cytochem (1973) 1.32
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
Experimental test of quantum contextuality in neutron interferometry. Phys Rev Lett (2009) 1.19
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
Changes in rat liver cells induced by orotic acid feeding. Am J Pharm Sci Support Public Health (1966) 1.12
Lysosomal defect of hepatic copper excretion in Wilson's disease (hepatolenticular degeneration). Gastroenterology (1973) 1.08
Orthotopic liver transplantation for Wilson's disease: a single-center experience. Transplantation (2001) 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
Studies on microperoxisomes. 3. Observations on human and rat hepatocytes. J Histochem Cytochem (1973) 1.05
Predictive testing for Wilson's disease using tightly linked and flanking DNA markers. Neurology (1991) 1.01
Wilson disease and idiopathic copper toxicosis. Am J Clin Nutr (1996) 1.00
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
Cytochemical studies of secretory and other granules associated with the endoplasmic reticulum in rat thyroid epithelial cells. Adv Cytopharmacol (1974) 0.97
Immunocytochemical localization of gamma-glutamyltransferase in rat kidney with protein A-horseradish peroxidase. Proc Natl Acad Sci U S A (1982) 0.96
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
Experimental demonstration of the stability of Berry's phase for a spin-1/2 particle. Phys Rev Lett (2009) 0.92
Hepatic metallothionein gene expression in toxic milk mice. J Nutr (1992) 0.91
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
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
Copper metallothionein in patients with hepatic copper overload. Eur J Clin Invest (1991) 0.89
Inverse relationship between net electric charge on the antigen and that on the sensitized cell in cellular immune response: demonstration with basic encephalitogen of the brain. J Exp Med (1975) 0.88
Spontaneous cholangiofibrosis in Long-Evans Cinnamon rats: a rodent model for Wilson's disease. Lab Anim Sci (1998) 0.88
Detection of multiple forms of human ceruloplasmin. A novel Mr 200,000 form. J Biol Chem (1990) 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
An array of mitochondrial alterations in the hepatocytes of Long-Evans Cinnamon rats. Hepatology (1995) 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
A prospective clinical trial of D-penicillamine in the treatment of primary biliary cirrhosis. Hepatology (1985) 0.85
Cytoplasmic crystals in human hepatocytes. Lab Invest (1971) 0.85
Penicillamine may detoxify copper in Wilson's disease. Lancet (1987) 0.85
Heavy metals and lysosomes. Front Biol (1976) 0.84
Off-diagonal geometric phase in a neutron interferometer experiment. Phys Rev Lett (2001) 0.84
Inherited copper toxicity of the liver in Bedlington terriers. Proc R Soc Med (1977) 0.84
Retinal pigment epithelium. Interrelations of endoplasmic reticulum and melanolysosomes in the black mouse and its beige mutant. Lab Invest (1979) 0.84
Lipolysosomes in human hepatocytes. Ultrastructural and cytochemical studies of patients with Wilson's disease. Lab Invest (1975) 0.84
Studies on microperoxisomes. IV. Interrelations of microperoxisomes, endoplasmic reticulum and lipofuscin granules. J Histochem Cytochem (1973) 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
Canine hepatic lysosomal copper protein: identification as metallothionein. Arch Biochem Biophys (1985) 0.82
Immunocytochemical localization of gamma-glutamyltransferase in induced hyperplastic nodules of rat liver. Proc Natl Acad Sci U S A (1983) 0.82
Defective urinary acidification in Wilson's disease. Ann Intern Med (1968) 0.82
Transport and intracellular distribution of copper in a human hepatoblastoma cell line, HepG2. Hepatology (1986) 0.82
Penicillamine-induced skin lesions. J Rheumatol Suppl (1981) 0.82
Continuous assessment of right ventricular ejection fraction: new pulmonary artery catheter versus transoesophageal echocardiography. Anaesthesia (2004) 0.81
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
Studies on the secretory process in exocrine pancreas cells. II. C57 black and beige mice. J Histochem Cytochem (1978) 0.80
The application of laser microprobe mass analysis to the study of biological material. Biometals (1996) 0.80
R-bodies of human rectal epithelial cells. Arch Pathol Lab Med (1976) 0.80
[Type A dissection. Principles of anesthesiological management]. Anaesthesist (2011) 0.80
The development of cirrhosis in Wilson's disease. Clin Gastroenterol (1975) 0.80
Neutron optical beam splitter from holographically structured nanoparticle-polymer composites. Phys Rev Lett (2010) 0.79
Prognosis of Wilsonian chronic active hepatitis. Gastroenterology (1991) 0.79