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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 13 (1990), S. 829-848 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The regulatory properties of human liver and muscle fructose-1, 6-bisphosphatases (FBPase) have been studied in control tissues obtained at autopsy and in tissues from a neonate with FBPase deficiency who died as a result of an overwhelming acidosis. Evidence is presented which suggests that the alkaline isoenzyme of FBPase, which is widely regarded as a laboratory artefact, may have an important rolein vivo in the regulation and control of glycolysis and gluconeogenesis. FBPase exhibits the hysteretic and dissociative properties associated with regulatory enzymes, and many of the factors which effect FBPase have inverse effects on phosphofructokinase activity, thus providing an integrated regulatory cycle for the control of the direction and rate of flux through the glycolytic pathway.
    Type of Medium: Electronic Resource
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