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  • 1
    ISSN: 1432-1076
    Schlagwort(e): Zellweger syndrome ; Prenatal diagnosis ; Phytanic acid ; Amniocytes ; Chorionic villous cells
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Normal amniocytes and chorionic villous cells in culture are able to produce14CO2 from exogenous [1-14C] phytanic acid. In contrast, cells from four fetuses at risk for the cerebro-hepato-renal (Zellweger) syndrome and related disorders showed a greatly reduced activity, indicating a block in oxidation of the fatty acid. Our data confirm that phytanic acid oxidase activity measurement can be used for the prenatal assessment of this group of disorders.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    ISSN: 1432-1076
    Schlagwort(e): Pristanic acid ; Phytanic acid ; Peroxisome ; β-oxidation
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The plasma of some patients with biochemical evidence of a generalised peroxisomal dysfunction (GPD) show greatly increased levels of phytanic acid as well as its α-oxidation product, pristanic acid (2, 6, 10, 14-tetramethylpentadecanoic acid). Increased amounts of 14- and 16- carbon branched chain fatty acids are also found in some of these patients. As pristanic acid is present in normal or near-normal amounts in classical Refsum disease and rhizomelic chondrodysplasia, two disorders characterised by deficiencies in phytanic acid oxidation, we speculate that its accumulation is not secondary to a defect in the α-oxidation of phytanic acid, but is indicative of a block in the peroxisomal β-oxidation of pristanic acid. The finding of phytanic acid, as well as a number of its metabolites in patients with inherited defects in peroxisomal biogenesis indicates that a number of the steps in phytanic acid degradation may be confined to peroxisomes.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    ISSN: 1432-1076
    Schlagwort(e): Infantile Refsum disease ; Phytanic acid ; Dietary treatment ; Peroxisomes ; Ultrastructure
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Two patients with infantile phytanic acid storage disease (infantile Refsum disease), one of whom showed the presence of morphologically normal peroxisomes in a liver biopsy, were treated with a low phytanic acid diet for more than 2 years and the effects of treatment on certain clinical, biochemical and ultrastructural parameters were examined. Both patients showed evidence of either an improvement or stabilisation in their clinical condition. Plasma phytanic acid levels decreased to near normal values in approximately 6 weeks after the introduction of the diet; plasma pipecolic acid also declined markedly but the decrease was not so rapid and its level remained abnormal. C26∶C22 fatty acid ratios decreased very slowly and even after 2 years the values remained grossly abnormal. Despite the marked reduction of phytanic acid in the liver, there was an increase in the C26∶C22 fatty acid ratios and this appeared to be paralleled by an increase in inclusion bodies. Our data suggest that some patients with the infantile form of Refsum disease may show some clinical benefit from dietary management and this is reflected biochemically by decreases in the plasma levels of phytanic acid and pipecolic acid.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    ISSN: 1432-0878
    Schlagwort(e): Median eminence ; Greenfinch (Chloris chloris L.) ; Anti-synthetic LHRH serum ; Binding sites ; Immunofluorescent localization
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Biologie , Medizin
    Notizen: Summary With an indirect immunofluorescence technique and an antiserum raised against synthetic LHRH, immunofluorescent granules were observed in fine radially oriented fibres in the palisade layer of the zona externa in both the anterior and the posterior divisions of the median eminence in the greenfinch. There was no specific immunofluorescence in the tractus supraoptico-hypophyseus. It is concluded that the greenfinch median eminence contains material which is similar to mammalian LHRH.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 5
    Digitale Medien
    Digitale Medien
    Springer
    Cell & tissue research 127 (1972), S. 552-569 
    ISSN: 1432-0878
    Schlagwort(e): Hypothalamus ; Median eminence ; Coturnix ; Tancyte and vascular patterns
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Biologie , Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung Im basalen Hypothalamus der Wachtel Coturnix wurde ein gut entwickeltes System von ependymalen Gliazellen mit langen Fortsätzen beobachtet. Diese Ependymzellen (Tanycyten, Horstmann, 1954) reichen bis an die Oberfläche des Gehirns. Die Tanycyten der Eminentia mediana und des ventro-lateralen Hypothalamus bilden eine Verbindung zwischen dem III. Ventrikel und dem portalen Hypophysenkreislauf. Die Fortsätze der ventro-lateralen Tanycyten enden in der Region des Sulcus infundibularis an einem lockeren Gefäßnetz, das sich in das primäre Kapillarnetz des portalen Gefäßsystems der Hypophyse fortsetzt. Das subependymale Kapillarnetz der Eminentia mediana verbindet die Gefäßsysteme der kontralateralen Hypothalamushälften. Hier gibt es keine direkten Verbindungen mit dem portalen Gefäßapparat. Aufgrund von licht- und elektronenmikroskopischen Studien wird das Ventrikelependym von Coturnix in eine dorsale „typische“ Region und in zwei „glanduläre“ Regionen (ventrolateral und ventral) gegliedert. Jedes dieser Gebiete zeigt unterschiedliche Tanycytenformen. Einer der beiden „glandulären“ Tanycytentypen (Typ 3), der im Verein mit dem ventrolateralen „glandulären“ Ependym auftritt, hat keinen Kontakt mit dem III. Ventrikel. Ultrastrukturstudien am „glandulären“ Ependym ergaben keine sicheren Unterschiede zwischen kastrierten und mit Oestrogen oder Testosteron behandelten, sowie zwischen geschlechtsreifen und nicht geschlechtsreifen Wachteln. Die Möglichkeit einer neuroendokrinen Funktion des Tanycyten-Gefäß-Systems wird diskutiert.
    Notizen: Summary A well developed system of ependymal glial cells with long basilar processes stretching to the surface of the brain (tanycytes, Horstmann, 1954) has been described in the basal hypothalamus of Coturnix quail. The tanycytes both in the median eminence and the ventro-lateral hypothalamus form a link between the third ventricle and the hypophysial circulation. The processes of the ventro-lateral tanycytes terminate in the region of the infundibular sulcus in apposition to a loose network of vessels which are continuous with the primary plexus of the hypophysial portal system. Within the median eminence, the subependymal capillary network connects the vasculature of the contra-lateral sides of hypothalamus. There are no direct connections with the hypophysial portal vessels. With the aid of the light and electron microscope the ventricular ependyma was divided into a dorsal “typical” region and two “glandular” regions (ventro-lateral and ventral). Each region contains different forms of tanycyte. One of the two forms of tanycyte (designated type 3) associated with the ventro-lateral glandular ependyma has no contact with the third ventricle. Ultrastructural studies on the “glandular” ependyma failed to show any obvious differences between castrated, oestrogen or testosterone implanted, and sexually mature or immature quail. The possibility that the tanycyte-vascular system may have a neuroendocrine role is discussed.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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