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Human microsomal glucose-6-phosphatase system

R C Nordlie1, K A Sukalski, W T Johnson

  • 1Department of Biochemistry and Molecular Biology, University of North Dakota School of Medicine, Grand Forks 58202.

European Journal of Pediatrics
|January 1, 1993
PubMed
Summary
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Glucose-6-phosphatase (G6Pase) is crucial for releasing liver glucose. Its deficiency causes glycogen storage disease type I, with researchers identifying two new variants (Ib and Ic).

Area of Science:

  • Biochemistry
  • Enzymology
  • Metabolic Disorders

Background:

  • Glucose-6-phosphatase (G6Pase) plays a key role in hepatic glucose release.
  • G6Pase deficiency is the identified cause of glycogenosis type I (von Gierke disease).

Purpose of the Study:

  • To briefly discuss the discovery and function of glucose-6-phosphatase.
  • To describe the identification of G6Pase deficiency in glycogenosis type I.
  • To present pioneering research characterizing G6Pase variants in glycogenosis types Ib and Ic.

Main Methods:

  • Literature review on G6Pase discovery and function.
  • Description of studies identifying G6Pase deficiency in glycogenosis.
  • Presentation of research characterizing G6Pase variants.

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Main Results:

  • Glucose-6-phosphatase (EC 3.1.3.9) is essential for glucose homeostasis.
  • Deficiency in G6Pase leads to glycogenosis type I.
  • Two variants, glycogenosis type Ib and Ic, were characterized.

Conclusions:

  • G6Pase is a multifunctional, multicomponent enzyme, particularly in the human liver.
  • Understanding G6Pase variants deepens insights into glycogen storage diseases.
  • Research highlights the enzyme's critical role in glucose metabolism and disease.