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  • 1
    Electronic Resource
    Electronic Resource
    Defective galactosylation in galactosemia: Is low cell UDPgalactose an explanation? (1995)
    Springer
    European journal of pediatrics 154 (1995), S. S65 
    Details
    ISSN: 1432-1076
    Keywords: Galatosylation ; Uridine diphosphate galactose ; Red blood cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract There is circumstantial evidence that defective galactosylation of complex glycoconjugates exists in tissues from galactosemic patients. Whether this is an etiologic factor in the long-term complications of the disorder is not known. Also not evident is the basis for the impaired galactosylation. The hypothesis that abnormally low cellular uridine diphosphate galactose (UDPgal) content is responsible has not been established. There is a tendency for galactosemic red cell UDPgal to be in the low normal range with a high uridine diphosphate glucose to UDPgal ratio. This may reflect an inability of red cell UDPgal-4′-epimerase to maintain a normal ratio and consequently higher levels of UDPgal. In the more complex white blood cells and cultured fibroblasts, the UDPgal content and the uridine diphosphate glucose to UDPgal ratio of galactosemics are normal. Therefore, defective galactosylation observed in galactosemic fibroblasts must result from a defect in the transfer of galactose from UDPgal to these moieties.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02143806
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    In utero galactose intoxication in animals (1995)
    Springer
    European journal of pediatrics 154 (1995), S. S82 
    Details
    ISSN: 1432-1076
    Keywords: Brain ; Cataract ; Enzyme induction ; Pregnant rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To assess in utero effects of galactose, diets containing large amounts of the sugar have been fed to pregnant rats with examination of the fetuses and newborns. Galactose levels in fetal blood parallel those of the dam and amniotic fluid contains high concentrations of galactose and galactitol. In the offspring and in the fetuses cataracts, and a decrease in brain weight, protein content and DNA are observed. There is an elvated content of galactitol and decrease in myo-inositol in the brain of galactose-intoxicated newborns. Brain synaptosomes of young rats whose mothers were fed galactose show a reduced response in vitro to acetylcholine as evidenced by a decrease in incorporation of myo-inositol into phosphatidylinositol and an inappropriate change in phosphatidylinositol content. The data indicate that there can be significant effects in the offspring of pregnant rats fed a high galactose diet. The findings support the concept that in utero toxicity may occur in the human galactosemic fetus.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02143810
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Galactosemia unsolved (1995)
    Springer
    European journal of pediatrics 154 (1995), S. S97 
    Details
    ISSN: 1432-1076
    Keywords: Chronic intoxication ; Complications ; Endogenous galactose ; Galactose metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Classic galactosemia is an enigmatic disorder that presents the challenge of unraveling the basis of the long-term complications of mental disability, speech defects, ovarian failure and neurologic syndromes which occur despite a galactose-restricted diet. A complete understanding of the pathobiochemistry and molecular genetics, and evaluation of the present theories for the poor long-term outcome, continuous intoxication, critical metabolite depletion and in utero damage is needed in order to design new therapeutic strategies. Answering this urgent question of how to treat galactosemic patients mandates enhanced clinical and basic research efforts.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02143813
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Concluding remarks (1995)
    Springer
    European journal of pediatrics 154 (1995), S. S106 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02143815
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    Paper (German National Licenses)
    Fulltext
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