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Related Experiment Videos

The glucose-6-phosphatase system.

Emile van Schaftingen1, Isabelle Gerin

  • 1Laboratoire de Chimie Physiologique, UCL and ICP, Avenue Hippocrate 75, B-1200 Brussels, Belgium. vanschaftingen@bchm.ucl.ac.be

The Biochemical Journal
|March 7, 2002
PubMed
Summary

Glucose-6-phosphatase (G6Pase) is a key enzyme for glucose production during starvation. Recent research supports a substrate-transport model for G6Pase activity, advancing our understanding of its function and related diseases.

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Area of Science:

  • Biochemistry
  • Molecular Biology
  • Enzymology

Background:

  • Glucose-6-phosphatase (G6Pase) is a crucial membrane-bound enzyme in the liver and kidneys, essential for maintaining blood glucose levels during fasting.
  • Early models proposed G6Pase as an unspecific enzyme requiring a specific glucose 6-phosphate translocase for substrate delivery to its lumenal catalytic site.
  • Alternative models suggested conformational changes influence G6Pase properties, contrasting with the substrate-transport hypothesis.

Purpose of the Study:

  • To review recent advancements in understanding the glucose 6-phosphate hydrolysis system.
  • To evaluate the evidence supporting different models of G6Pase function.
  • To highlight progress in identifying genetic mutations and developing inhibitors related to G6Pase and its translocase.

Main Methods:

  • Review of genetic studies, including cloning of genes for G6Pase, glucose 6-phosphate translocase, and a related protein.
  • Analysis of findings from synthesized and isolated specific inhibitors of G6Pase and its translocase.
  • Examination of research on the regulation of G6Pase gene expression by hormones and metabolites.

Main Results:

  • Cloning of genes for G6Pase and glucose 6-phosphate translocase, with mutations identified in glycogen storage diseases Ia and Ib.
  • Development of specific inhibitors provides further support for the substrate-transport model.
  • Significant progress in understanding the regulation of G6Pase expression by insulin, glucocorticoids, cAMP, and glucose.

Conclusions:

  • Current evidence strongly supports the substrate-transport model for G6Pase function, initially proposed by Arion.
  • Genetic and pharmacological studies have significantly advanced the understanding of G6Pase and its associated translocase.
  • Further research into G6Pase regulation and its role in metabolic diseases continues to be an active area.

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