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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Mineralogy and petrology 40 (1989), S. 173-182 
    ISSN: 1438-1168
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences
    Notes: Summary The Chiang Khan meteorite fell on 18th November, 1981 at Chiang Khan, Thailand. It consists of olivine, orthopyroxene, clinopyroxene, Fe-Ni metal, troilite, chromite, plagioclase, glass, and phosphate in order of abundance. Olivine forms barred or porphyritic chondrules, and its composition is uniform (average Fo80.2), close to the average composition of olivine in equilibrated H chondrites. Orthopyroxene and clinopyroxene also have compositions similar to those in equilibrated H chondrites. Both well-defined chondrules and their broken fragments are present in the recrystallized matrix. Microcrystalline plagioclase and clinopyroxene often occur in the groundmass of chondrules, but clear interstitial plagioclase is absent. Chemical composition of chromite plots in the field of chromites in H chondrites. Chiang Khan meteorite is thus classified as an equilibrated H 5 type chondrite. The equilibrium temperatures estimated by using mineral pairs are as follows: Opx-Cpx 800–900°C; Ol-Chromite 510°C. Water content is 0.24 wt %, and the hydrogen isotopic composition (ΔD) is −89.5‰ In the thermal demagnetization experiment magnetization steadily decreased from 0 to 500°C, whereas the remanent magnetization obtained in the A.C. demagnetization experiment is very unstable, probably owing to the large grain size of the Fe-Ni metal.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 19 (1989), S. 474-476 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A rare case of a primary malignant lymphoma of the rectum in a 7 year old girl is presented. Although rectal polyps in children are usually benign juvenile polyps, a rare possibility of malignancy should be kept in mind. An adequate histological examination is essential.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0428
    Keywords: Anti-insulin antibody ; insulin receptor ; insulin binding ; cross-linking ; disuccinimidyl suberate ; Fcy receptor ; liver membrane
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In the presence of anti-insulin antibody, 2- to 3-fold enhancement of 125I-insulin binding to liver membranes was observed when binding was estimated by the radioactivity of 125I-insulin bound to the membrane pellets. However, after 125'I-insulin was covalently cross-linked to liver membranes using disuccinimidyl suberate in the presence of anti-insulin antibody, sodium dodecyl sulfate-polyacrylamide gel electrophoresis and autoradiography showed that 125I-insulin bound to the α-subunit of the insulin receptor was inhibited by antiinsulin antibody in an dose-dependent manner. More importantly, at an anti-insulin antibody dilution range between 1:50 and 1:5,000, sodium dodecyl sulfate-polyacrylamide gel electrophoresis revealed two 125I-labelled bands of mol wt 62,000 and 27,000, while only one band of mol wt 130,000 was revealed in the absence of anti-insulin antibody. These Mr=62,000 and Mr=27,000 bands were found to be the heavy and the light chain of anti-insulin IgG molecules respectively. Pepsin digested anti-insulin serum had only an inhibitory effect on 125I-insulin binding to liver membranes. Non-immunized guinea pig serum or IgG completely abolished the enhanced effect of anti-insulin antibody. Further, this enhanced effect was inhibited by Fc fragment-specific anti-IgG serum or H&L-chain-specific anti-IgG serum in a dosedependent manner. Protein A also inhibited the effect of antiinsulin antibody. In IM-9 lymphocytes and human red blood cell ghosts, which have no Fcy receptors, enhancement of insulin binding was not observed in the presence of anti-insulin antibody. These data suggest that anti-insulin antibody-induced enhancement of insulin binding to liver membranes is not due to the enhanced binding to the insulin receptor itself but probably due to the binding of insulin-anti-insulin antibody complex to the Fcγ receptor.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Homocystinuria ; Homocystine ; Methionine ; Cystathionine synthase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A boy with homocystinuria due to cystathionine synthase deficiency was found to have hypermethioninaemia by neonatal blood screening, but was not diagnosed as homocystinuric until 3 months of age because urinary homocystine was not detected by the cyanidenitroprusside test or on two examinations with a sensitive amino acid autoanalyser. These findings indicate that tests for urinary homocystine should be made repeatedly with an amino acid auto-analyser in newborn infants with hypermethioninaemia until the enzyme defect is identified.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Vitamin-D-dependent rickets type II ; 1,25-Dihydroxyvitamin D receptor ; Alopecia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Rickets in a 3-year-old boy and his 1-year-old sister, both with alopecia, was cured by treatment with 50,000IU of vitamin D2 daily for 2 years and did not recur within 14 years after cessation of therapy. A diagnosis of vitamin-D-dependent rickets type II was made in these patients at the ages of 20 and 18 years based on the findings that 1,25-dihydroxyvitamin D3 [1,25 (OH)2D3] did not inhibit DNA biosynthesis in phytohaemagglutinin-stimulated lymphocytes and that cultured skin fibroblasts showed impaired nuclear uptake and normal cytosol binding of [3H] 1,25(OH)2D3. Surprisingly, the serum 1,25(OH)2D levels of these patients were high and their serum 24,25-dihydroxyvitamin D levels were low, although neither patient showed any symptoms except alopecia. The presence of vitamin D metabolite imbalances in the absence of rickets in these patients might be explained by differences in sensitivity to 1,25(OH)2D3 of bone formation and vitamin D metabolism. In addition, changes of sensitivity to treatment with vitamin D derivatives might be a consequence of differentiation of target cells. From the present findings, it is suggested that in this disease treatment with a sufficient dose of vitamin D derivatives should be initiated in the active phase of rickets.
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  • 6
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sodium dichloroacetate (DCA) was administered orally at a dose of 50 mg per kg body weight twice or three times per day to a newborn infant with lactic acidosis of unknown cause (patient 1) and to a 15-year-old boy with mitochondrial encephalomyopathy associated with lactic acidosis (patient 2). In patient 1, during treatment with DCA, DCA accumulated in the blood judging from the findings that the urinary excretion of DCA increased cumulatively and the blood lactate level rapidly decreased to the normal range. In patient 2, the blood DCA level gradually increased during treatment to a concentration of 250 µgml−1 and the blood lactate level decreased and was maintained within the normal range. DCA was detected in the brain (25 µg g tissue−1) and the liver, kidney and muscle (33.8, 33.8 and 26.3 µg g tissue−1, respectively) obtained at autopsy of patient 1, and in the cerebrospinal fluid of patient 2 at a concentration of 125 µg ml−1 when the blood concentration was 250 µg ml−1. The lactate levels in the cerebrospinal fluid decreased from 7 and 4mmoll−1 to 2.4 and 2.6 mmoll−1 in patients 1 and 2, respectively. Thus DCA may be useful in clinical treatment of chronic congenital lactic acidosis because it seems to cross the blood-brain barrier. However, it must be given at non-toxic doses, determined by monitoring the concentrations of lactate and DCA in the blood, because orally administered DCA tends to accumulate in tissues.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 8 (1985), S. 177-177 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 12 (1989), S. 379-385 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The biochemical nature of the pyruvate dehydrogenase complex (PDHC) in muscle was studied in a patient with pyruvate dehydrogenase complex deficiency. The enzyme activity was approximately 30% of the control level and the apparentK m value of the enzyme was similar to the control value. The immunoblot pattern of each subunit protein, E1α, E1β, the component X, E2 and E3, was comparable to that of the control on both one-and two-dimensional electrophoresis, the staining of each subunit protein being reduced in intensity, corresponding to the reduced enzyme activity. The enzyme deficiency is likely to be quantitative rather than qualitative, although the actual mechanism is unknown.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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