Studies on copper metabolism. XIV. Radioactive copper studies in normal subjects and in patients with hepatolenticular degeneration.

نویسندگان

  • J A BUSH
  • J P MAHONEY
  • H MARKOWITZ
  • C J GUBLER
  • G E CARTWRIGHT
  • M M WINTROBE
چکیده

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منابع مشابه

Studies with radioactive copper ( 64 Cu and 67 Cu); the incorporation of radioactive copper into caeruloplasmin in Wilson's disease and in primary biliary cirrhosis.

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Reports have appeared of abnormal copper metabolism in retinitis pigmentosa, and of a family with vitelliform retinal degeneration in which other members suffered from hepatolenticular degeneration. In the present study 15 patients with retinitis pigmentosa, 4 with various other retinal degenerations, and 1 with a family disposition to retinitis pigmentosa were examined. The copper concentratio...

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Clinically and electrophysiologically confirmed cases of primary retinitis pigmentosa have been investigated regarding their copper metabolic state. It is observed that these patients show a normal or near normal serum copper concentration, very low plasma caeruloplasmin concentration, and a very high copper urinary excretion. A similarity between this condition and hepatolenticular degeneratio...

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Observations on ceruloplasmin in Wilson's disease.

In the light of present knowledge hepatolenticular degeneration is classified as a metabolic disease, probably based on an inborn error of metabolism and transmitted by an autosomal recessive gene. The biochemical manifestations of Wilson's disease have been studied extensively and were summarized in several recent reviews (1-4). The findings point to aberrations in copper and protein metabolis...

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Copper metabolism in normal adults and in clinically normal relatives of patients with Wilson's disease.

Wilson's disease (hepato-lenticular degeneration) is now known to be associated with an inborn error of metabolism. The following biochemical abnormalities have been repeatedly confirmed in patients suffering from the disease (Scheinberg, 1956; Bickel, Neale, and Hall, 1957): Increased content of copper in the liver, brain, and other tissues, increased urinary excretion of copper, a decreased l...

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عنوان ژورنال:
  • The Journal of clinical investigation

دوره 34 12  شماره 

صفحات  -

تاریخ انتشار 1955