Literature DB >> 3569749

A copper-sulfur complex in the liver of a patient with Wilson's disease.

A Sasa, H Hayashi, A Yagi, S Ohguchi, R Kidokoro, Y Sato, N Sakamoto.   

Abstract

An asymptomatic 16-year-old boy was found to have Wilson's disease without Kayser-Fleischer rings. Liver biopsy showed chronic active hepatitis with 1025 micrograms copper/g dry weight. After 19 months of d-penicillamine therapy, the liver histology became almost normal and the copper content decreased to 238 micrograms/g dry weight. The liver specimens obtained before and after treatment were studied by X-ray probe microanalysis. After treatment, both copper and sulfur decreased in hepatocellular lysosomes. The estimated molar ratio of the decreased copper to the decreased sulfur was 32/100. These figures suggest that lysosomal copper exists in the form of metallothionein.

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Year:  1986        PMID: 3569749     DOI: 10.1007/bf02774493

Source DB:  PubMed          Journal:  Gastroenterol Jpn        ISSN: 0435-1339


  13 in total

1.  Metallothionein: a cadmium- and zinc-containing protein from equine renal cortex.

Authors:  J H KAGI; B L VALEE
Journal:  J Biol Chem       Date:  1960-12       Impact factor: 5.157

2.  Penicillamine, a new oral therapy for Wilson's disease.

Authors:  J M WALSHE
Journal:  Am J Med       Date:  1956-10       Impact factor: 4.965

3.  Human hepatic metallothioneins.

Authors:  R H Bühler; J H Kägi
Journal:  FEBS Lett       Date:  1974-02-15       Impact factor: 4.124

4.  Long-term therapy of Wilson's disease.

Authors:  A Deiss; R E Lynch; G R Lee; G E Cartwright
Journal:  Ann Intern Med       Date:  1971-07       Impact factor: 25.391

5.  Hepatic copper distribution in primary biliary cirrhosis shown by the scanning proton microprobe.

Authors:  D J Vaux; F Watt; G W Grime; J Takacs
Journal:  J Clin Pathol       Date:  1985-06       Impact factor: 3.411

6.  Asymptomatic primary biliary cirrhosis. Presentation, histology, and results with D-penicillamine.

Authors:  C R Fleming; J Ludwig; E R Dickson
Journal:  Mayo Clin Proc       Date:  1978-09       Impact factor: 7.616

7.  Orcein positive granules in the hepatocytes in chronic intrahepatic cholestasis. Morphological, histochemical and electron X-ray microanalytical examination.

Authors:  Y Nakanuma; T Karino; G Ohta
Journal:  Virchows Arch A Pathol Anat Histol       Date:  1979-05-14

8.  Hepatic copper in primary biliary cirrhosis: biliary excretion and response to penicillamine treatment.

Authors:  M P Salaspuro; P Pikkarainen; P Sipponen; E Vuori; T A Miettinen
Journal:  Gut       Date:  1981-11       Impact factor: 23.059

9.  Electron probe X-ray analysis on human hepatocellular lysosomes with copper deposits: copper binding to a thiol-protein in lysosomes.

Authors:  T Hanaichi; R Kidokoro; H Hayashi; N Sakamoto
Journal:  Lab Invest       Date:  1984-11       Impact factor: 5.662

10.  A technique for ultracryotomy of cell suspensions and tissues.

Authors:  K T Tokuyasu
Journal:  J Cell Biol       Date:  1973-05       Impact factor: 10.539

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  1 in total

1.  Hepatocellular copper toxicity and its attenuation by zinc.

Authors:  M L Schilsky; R R Blank; M J Czaja; M A Zern; I H Scheinberg; R J Stockert; I Sternlieb
Journal:  J Clin Invest       Date:  1989-11       Impact factor: 14.808

  1 in total

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