Library

feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    ISSN: 1432-1076
    Keywords: Pristanic acid ; Phytanic acid ; Peroxisome ; β-oxidation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 2
    ISSN: 1432-1076
    Keywords: Zellweger syndrome ; Prenatal diagnosis ; Phytanic acid ; Amniocytes ; Chorionic villous cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 3
    ISSN: 1432-1076
    Keywords: Infantile Refsum disease ; Phytanic acid ; Dietary treatment ; Peroxisomes ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 4
    ISSN: 1432-0878
    Keywords: Osmoregulation ; fos immunocytochemistry ; Hypothalamus ; Vasotocin ; Domestic hen Gallus domesticus ; Japanese quail Coturnix japonica ; Ring dove Streptopelia risoria ; Zebra finch, Taenopygia guttata
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Domestic hens were injected intraperitoneally with hypertonic or isotonic saline and killed 0.5, 1, 2, 6, 12 and 24 h later. Japanese quail, Ring doves and Zebra finches were treated in the same way and killed 2 h later. Using fos immunocytochemistry, fos-positive cells were visualized in the preoptic-anterior hypothalamus. In all species, two hours after treatment with hypertonic but not with isotonic saline, a prominent cluster of fos-positive cells was seen close to the mid-line, dorsal to the anterior part of the third ventricle, in and around the nucleus commissurae pallii. The cell cluster was associated with the dorsal region of the organum vasculosum laminae terminalis and passed caudo-dorsally above the anterior commissure into the area of the subfornical organ, spreading diffusely into the nucleus septalis medialis and the nucleus dorsomedialis anterior thalami. The maximal expression of c-fos was seen 2 h after treatment with hypertonic saline: weak fos immunoreactive product was seen at 0.5, 1 h and 6 h but not after 12 and 24 h. In all birds, 2 h after treatment with hypertonic but not with isotonic saline, fos-positive cells were also seen in the nucleus paraventricularis and nucleus supraopticus. Double immunocytochemistry in the domestic hen with an antibody to vasotocin showed that these fos-positive cells were classical magnocellular vasotocinergic neurones. This study extends earlier studies in birds using lesioning and electrophysiological techniques to identify the precise cellular localization of the avian “osmoreceptive complex” projected onto a stereotaxic atlas.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 5
    ISSN: 1432-0878
    Keywords: Key words: Osmoregulation ; fos immunocytochemistry ; Hypothalamus ; Vasotocin ; Domestic hen Gallus domesticus ; Japanese quail Coturnix japonica ; Ring dove Streptopelia risoria ; Zebra finch ; Taenopygia guttata
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. Domestic hens were injected intraperitoneally with hypertonic or isotonic saline and killed 0.5, 1, 2, 6, 12 and 24 h later. Japanese quail, Ring doves and Zebra finches were treated in the same way and killed 2 h later. Using fos immunocytochemistry, fos-positive cells were visualized in the preoptic-anterior hypothalamus. In all species, two hours after treatment with hypertonic but not with isotonic saline, a prominent cluster of fos-positive cells was seen close to the mid-line, dorsal to the anterior part of the third ventricle, in and around the nucleus commissurae pallii. The cell cluster was associated with the dorsal region of the organum vasculosum laminae terminalis and passed caudo-dorsally above the anterior commissure into the area of the subfornical organ, spreading diffusely into the nucleus septalis medialis and the nucleus dorsomedialis anterior thalami. The maximal expression of c-fos was seen 2 h after treatment with hypertonic saline: weak fos immunoreactive product was seen at 0.5, 1 h and 6 h but not after 12 and 24 h. In all birds, 2 h after treatment with hypertonic but not with isotonic saline, fos-positive cells were also seen in the nucleus paraventricularis and nucleus supraopticus. Double immunocytochemistry in the domestic hen with an antibody to vasotocin showed that these fos-positive cells were classical magnocellular vasotocinergic neurones. This study extends earlier studies in birds using lesioning and electrophysiological techniques to identify the precise cellular localization of the avian ”osmoreceptive complex” projected onto a stereotaxic atlas.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...