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Characterization of human fructose-1,6-bisphosphatase in control and deficient tissues

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Journal of Inherited Metabolic Disease

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.

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Authors and Affiliations

  1. Department of Clinical Biochemistry, Royal Children's Hospital, Flemington Road, Parkville, 3052, Melbourne, Australia

    A. Adams & C. Redden

  2. Department of Cardiology, Royal Children's Hospital, Flemington Road, Parkville, 3052, Melbourne, Australia

    S. Menahem

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  1. A. Adams
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  2. C. Redden
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  3. S. Menahem
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Adams, A., Redden, C. & Menahem, S. Characterization of human fructose-1,6-bisphosphatase in control and deficient tissues. J Inherit Metab Dis 13, 829–848 (1990). https://doi.org/10.1007/BF01800207

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  • DOI: https://doi.org/10.1007/BF01800207

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