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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 137 (1981), S. 17-21 
    ISSN: 1432-1076
    Keywords: Menkes kinky hair syndrome ; Copper and zinc contents ; Copper and zinc proteins ; Metalloprotein ; Metallothionein-like protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Copper and zinc concentrations, and the nature of the copper- and zinc-binding proteins, were studied using tissues from a Menkes patient who had been given intravenous infusions of cupric acetate. The liver and brain copper contents were lower than in an untreated, non-Menkes control, and the spleen, intestine and kidney showed higher copper concentrations than control tissues. Zinc concentrations in all the organs (except the kidneys) from the Menkes patient were slightly lower than those the control child. Using Sephadex G-75 column chromatography of cytosols (105,000 x g supernatant), three copper- and zinc-containing peaks were eluted. In all the Menkes tissues studied, copper was prominent in peak 3. On the other hand, peak 3 was the smallest and peak 1 was the largest in the control tissues. Zinc predominated in peak 1 in both Menkes and control tissues, except for Menkes kidney. In this tissue peak 3 was again the largest. The copper- and zinc-binding material in peak 3 fractions from Menkes kidney was chromatographed on DEAE-cellulose columns. Three copper- and zinc-containing peaks were observed at the same positions as metallothionein from human adult kidneys.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 134 (1980), S. 205-210 
    ISSN: 1432-1076
    Keywords: Menkes kinky hair syndrome ; Menkes fibroblasts ; Copper metabolism ; Metalloprotein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Menkes fibroblast cultures were established and copper metabolism was investigated. Menkes fibroblasts contained four to six times higher concentrations of copper than control cells. In Menkes cells more than 90% of the intracellular copper was present in cytosol (105,000 x g supernatant fraction); in control fibroblasts the corresponding value was about 67%. During cultivation of fibroblasts in medium supplemented with 100 ng/ml of copper (270 ng CuCl2·2H2O /ml), the amount of copper increased continuously in Menkes cells, at least up to 4 days, while in control cells in reached a maximum after 24 h, followed by a gradual decrease. When the medium was replaced with one without copper chloride, copper concentrations in Menkes cells returned to the original level in three days, whereas those in control cells returned to the normal level in one day. Using Sephadex G-75 column chromatography of cytosol, two copper-containing peaks were observed (peaks 1 and 3, corresponding to the peaks from rat liver cytosol). Approximately 75% of the copper in the cytosol from Menkes cells was eluted in peak 3. The corresponding copper peak was very small in control cells. Copper peaks 1 and 3 from both cells increased after treating cells with copper chloride and the increase was inhibited by cycloheximide, an inhibitor of protein biosynthesis. Metallothionein purified from human kidneys was eluted as a single copper-protein from a Sephadex G-75 column in the same fractions as peak 3. SDS polyacrylamide-slab gel electrophoresis of the purified metallo-thionein and the material eluted in peak 3 from Menkes fibroblasts showed single peaks for copper at identical migration distances.
    Type of Medium: Electronic Resource
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