The biochemistry and molecular biology of the glucose-6-phosphatase system

Exp Biol Med (Maywood). 2002 Sep;227(8):601-8. doi: 10.1177/153537020222700807.

Abstract

Progress has continued to be made over the past 4 years in our understanding of the glucose-6-phosphatase (G6Pase) system. The gene for a second component of the system, the putative glucose-6-P transporter (G6PT), was cloned, and mutations in this gene were found in patients diagnosed with glycogen storage disease type 1b. The functional characterization of this putative G6PT has been initiated, and the relationship between substrate transport via the G6PT and catalysis by the system's catalytic subunit continues to be explored. A lively debate over the feasibility of various aspects of the two proposed models of the G6Pase system persists, and the functional/structural relationships of the individual components of the system remain a hot topic of interest in G6Pase research. New evidence supportive of physiologic roles for the biosynthetic functions of the G6Pase system in vivo also has emerged over the past 4 years.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Antiporters / genetics
  • Antiporters / physiology
  • Catalysis
  • Catalytic Domain
  • Diabetes Mellitus, Type 2 / enzymology
  • Glucose / metabolism
  • Glucose-6-Phosphatase / chemistry
  • Glucose-6-Phosphatase / genetics
  • Glucose-6-Phosphatase / physiology*
  • Glucose-6-Phosphate / metabolism
  • Glycogen Storage Disease Type I / enzymology
  • Glycogen Storage Disease Type I / genetics
  • Humans
  • Mice
  • Mice, Knockout
  • Models, Molecular
  • Molecular Sequence Data
  • Monosaccharide Transport Proteins / genetics
  • Monosaccharide Transport Proteins / physiology
  • Phosphates / metabolism
  • Phosphorylation
  • Protein Conformation
  • Protein Subunits
  • Recombinant Fusion Proteins / physiology
  • Structure-Activity Relationship

Substances

  • Antiporters
  • Monosaccharide Transport Proteins
  • Phosphates
  • Protein Subunits
  • Recombinant Fusion Proteins
  • SLC37A4 protein, human
  • Slc37a4 protein, mouse
  • glucose 6-phosphate(transporter)
  • Glucose-6-Phosphate
  • Glucose-6-Phosphatase
  • Glucose