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  • 1980-1984  (3)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular evolution 15 (1980), S. 347-354 
    ISSN: 1432-1432
    Keywords: Adenosine 5′-phosphorimidazolide ; Template-directed condensation ; Oligoadenylates ; Adsorption ; Hydroxyapatite ; Prebiotic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Summary Polyuridylic acid is adsorbed completely from aqueous solution by hydroxyapatite under conditions that permit template-directed synthesis of oligoadenylates in free solution. The yield of oligoadenylates is enhanced to almost the same extent by poly(U) in the presence or the absence of hydroxyapatite. Under very similar conditions small quantities of hydroxyapatite adsorb higher molecular-weight oligoadenylates selectively from a mixture of oligomers. On the basis of these results we propose a mechanism for prebiotic oligonucleotide formation in which selective adsorption on hydroxyapatite or some other immobilized anion-exchanging material plays a major role. Monomers are released from the surface for reactivation, while oligomers are retained in a protected environment by adsorption to the apatite surface.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 4 (1981), S. 191-195 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Enzyme assays for phenylalanine mono-oxygenase have been performed on a variety of non-hepatic human cell types under varying conditions. The cell types studied were cultured fibroblasts, short-term lymphocyte cultures, long-term lymphoblastoid cultures, cultured amniotic fluid cells, hair roots and placental extracts. The cultured cells were assayed after growth under standard conditions and in the presence of a high concentration of substrate, a low concentration of end-product, added hydrocortisone or dexamethasone and under combinations of these conditions. In no instance was a significant enzyme activity obtained.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 6 (1983), S. 62-81 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This paper reports the clinical and biochemical results in six patients with Hurler disease (Mucopolysaccharidosis IH; McKusick 25280), two patients with Hunter disease (Mucopolysaccharidosis II; McKusick 25285) and one patient with Sanfilippo B disease (Mucopolysaccharidosis IIIB; McKusick 25292) who were treated by fibroblast transplantation. Except for one patient who died for a coincidental reason, the patients have been studied for between 2.5 and 4.5 years. The clinical course of the disease was not materially altered. There was no evidence that the patients had developed immune responses against the transplanted fibroblasts. Transplantation did not produce measurable levels of either α-l-iduronidase (EC 3.2.1.76) in the leukocytes from patients with Hurler disease or ofN-acetyl-α-d-glucosaminidase (EC 3.2.1.50) in the plasma of the patient with Sanfilippo B disease. Under the conditions used for the assay, leukocytes from the patients with Hunter disease had detectable levels of residual α-l-idurono-2-sulphate sulphatase activity which were increased after the transplants, although these changes were of inconstant size and their time course was not consistently related to the transplantations. Cytogenetic studies in cases where the donor was of the opposite sex detected only cells of the recipient's sex among the fibroblasts grown from biopsies of the transplantation sites. The technique used would have detected a donor to recipient cell ratio of 1:100. We found no consistent long-term trends in the excretion patterns of glycosaminoglycans and oligosaccharides from either a quantitative or qualitative point of view which could be specifically related to the transplantation. The combined administration of immunosuppressive doses of prednisolone and azathioprine was associated with an increased excretion of the lower molecular weight glycosaminoglycans. We conclude that fibroblast transplantation is not therapeutically useful in the diseases studied.
    Type of Medium: Electronic Resource
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