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Gene Review

SLC37A4  -  solute carrier family 37 (glucose-6...

Homo sapiens

Synonyms: G6PT, G6PT1, G6PT2, G6PT3, GSD1b, ...
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Disease relevance of SLC37A4


Psychiatry related information on SLC37A4


High impact information on SLC37A4


Chemical compound and disease context of SLC37A4


Biological context of SLC37A4

  • G6Pase and G6PT work in concert to maintain glucose homeostasis in gluconeogenic organs [9].
  • Rapid confirmation of clinically suspected diagnosis of GSD 1, reliable carrier testing, and prenatal diagnosis are facilitated by mutation analyses of the chromosome 11-bound G6PT gene as well as the chromosome 17-bound G6Pase gene [9].
  • Whereas G6Pase is exclusively expressed in gluconeogenic cells, G6PT is ubiquitously expressed and its deficiency generally causes a more severe phenotype [9].
  • A mRNA leucocyte G6PT deficiency has been suggested to account for the glucose phosphorylation and subsequent calcium sequestration defects observed in theses cells [10].
  • Here, we report on the analysis by single-stranded conformation polymorphism (SSCP) and/or DNA sequencing of the exons of the G6PT in 14 patients diagnosed either as affected by the GSD 1b or 1c subtypes [11].

Anatomical context of SLC37A4

  • Both G6Pase and G6PT are associated with the endoplasmic reticulum (ER) membrane [12].
  • Using amino- and carboxyl-terminal tagged G6PT, we demonstrate that proteolytic digestion of intact microsomes resulted in the cleavage of both tags, indicating that both termini of G6PT face the cytoplasm [1].
  • Quantitative analysis of glucose-6-phosphate translocase gene expression in various human tissues and haematopoietic progenitor cells [3].
  • Further, we show that G6PT mRNA is expressed in all organs and tissues examined, but that the vG6PT transcript is expressed exclusively in the brain, heart, and skeletal muscle [13].
  • Conversely, expression of both the mouse homologue of the previously-described brain-specific G6P translocase T1 (G6PT1) variant and of the hepatic G6PT1 isoform was easily detectable in hypothalamus and cortex tissues [14].

Associations of SLC37A4 with chemical compounds

  • GSD 1 non-a is characterized by defective microsomal glucose-6-phosphate or pyrophosphate/phosphate transport due to mutations in G6PT (glucose-6-phosphate translocase gene) encoding a microsomal transporter protein [9].
  • It was suggested that GSD1b patients suffered from a G6P transporter (G6PT) defect and the first mutation in the G6PT gene subsequently recognised [10].
  • To determine which of these two models is correct, we generated two G6PT mutants, T53N and S55N, that created a potential Asn-linked glycosylation site at residues 53-55 (N53SS) or 55-57 (N55QS), respectively [1].
  • A region of G6PT corresponding to amino acid residues 50-71, which constitute a transmembrane segment in the twelve-domain model, are situated in a 51-residue luminal loop in the ten-domain model [1].
  • Whereas wild-type G6PT is not a glycoprotein, both T53N and S55N mutants are glycosylated, strongly supporting the ten-helical model for G6PT [1].

Other interactions of SLC37A4


Analytical, diagnostic and therapeutic context of SLC37A4


  1. Transmembrane topology of human glucose 6-phosphate transporter. Pan, C.J., Lin, B., Chou, J.Y. J. Biol. Chem. (1999) [Pubmed]
  2. Homology modeling of the human microsomal glucose 6-phosphate transporter explains the mutations that cause the glycogen storage disease type Ib. Almqvist, J., Huang, Y., Hovmöller, S., Wang, D.N. Biochemistry (2004) [Pubmed]
  3. Quantitative analysis of glucose-6-phosphate translocase gene expression in various human tissues and haematopoietic progenitor cells. Ihara, K., Nomura, A., Hikino, S., Takada, H., Hara, T. J. Inherit. Metab. Dis. (2000) [Pubmed]
  4. Silencing of the human microsomal glucose-6-phosphate translocase induces glioma cell death: potential new anticancer target for curcumin. Belkaid, A., Copland, I.B., Massillon, D., Annabi, B. FEBS Lett. (2006) [Pubmed]
  5. The chemopreventive properties of chlorogenic acid reveal a potential new role for the microsomal glucose-6-phosphate translocase in brain tumor progression. Belkaid, A., Currie, J.C., Desgagnés, J., Annabi, B. Cancer Cell Int. (2006) [Pubmed]
  6. How many forms of glycogen storage disease type I? Veiga-da-Cunha, M., Gerin, I., Van Schaftingen, E. Eur. J. Pediatr. (2000) [Pubmed]
  7. Apoptotic neutrophils in the circulation of patients with glycogen storage disease type 1b (GSD1b). Kuijpers, T.W., Maianski, N.A., Tool, A.T., Smit, G.P., Rake, J.P., Roos, D., Visser, G. Blood (2003) [Pubmed]
  8. A direct method for the diagnosis of human hepatic type 1b and type 1c glycogen-storage disease. Waddell, I.D., Hume, R., Burchell, A. Clin. Sci. (1989) [Pubmed]
  9. Molecular genetics of type 1 glycogen storage disease. Janecke, A.R., Mayatepek, E., Utermann, G. Mol. Genet. Metab. (2001) [Pubmed]
  10. Historical highlights and unsolved problems in glycogen storage disease type 1. Moses, S.W. Eur. J. Pediatr. (2002) [Pubmed]
  11. Mutations in the glucose-6-phosphate transporter (G6PT) gene in patients with glycogen storage diseases type 1b and 1c. Galli, L., Orrico, A., Marcolongo, P., Fulceri, R., Burchell, A., Melis, D., Parini, R., Gatti, R., Lam, C., Benedetti, A., Sorrentino, V. FEBS Lett. (1999) [Pubmed]
  12. Type I glycogen storage diseases: disorders of the glucose-6-phosphatase complex. Chou, J.Y., Matern, D., Mansfield, B.C., Chen, Y.T. Curr. Mol. Med. (2002) [Pubmed]
  13. Human variant glucose-6-phosphate transporter is active in microsomal transport. Lin, B., Pan, C.J., Chou, J.Y. Hum. Genet. (2000) [Pubmed]
  14. Expression of glucose-6-phosphatase system genes in murine cortex and hypothalamus. Goh, B.H., Khan, A., Efendić, S., Portwood, N. Horm. Metab. Res. (2006) [Pubmed]
  15. The glucose-6-phosphatase system. van Schaftingen, E., Gerin, I. Biochem. J. (2002) [Pubmed]
  16. Amelioration of neutrophil membrane function underlies granulocyte-colony stimulating factor action in glycogen storage disease 1b. Lesma, E., Riva, E., Giovannini, M., Di Giulio, A.M., Gorio, A. International journal of immunopathology and pharmacology. (2005) [Pubmed]
  17. Predicting the three-dimensional structure of the human facilitative glucose transporter glut1 by a novel evolutionary homology strategy: insights on the molecular mechanism of substrate migration, and binding sites for glucose and inhibitory molecules. Salas-Burgos, A., Iserovich, P., Zuniga, F., Vera, J.C., Fischbarg, J. Biophys. J. (2004) [Pubmed]
  18. Prenatal diagnosis of glycogen storage disease type 1b using denaturing high performance liquid chromatography. Lam, C.W., Sin, S.Y., Lau, E.T., Lam, Y.Y., Poon, P., Tong, S.F. Prenat. Diagn. (2000) [Pubmed]
  19. Assignment1 of glucose 6-phosphate translocase (G6PT1) to human chromosome band 11q23.3 by in situ hybridization. Ihara, K., Takabayashi, A., Terasaki, K., Hara, T. Cytogenet. Cell Genet. (1998) [Pubmed]
  20. Disturbed lipid metabolism in glycogen storage disease type 1. Bandsma, R.H., Smit, G.P., Kuipers, F. Eur. J. Pediatr. (2002) [Pubmed]
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