glycerol 3 phosphate dehydrogenase


Gene Symbol: glycerol 3 phosphate dehydrogenase
Description: glycerol phosphate dehydrogenase 2, mitochondrial
Alias: AA408484, AI448216, AU021455, AW494132, GPDH, Gdm1, Gpd-m, Gpdh-m, TISP38, glycerol-3-phosphate dehydrogenase, mitochondrial, FAD-linked glycerol-3-phosphate dehydrogenase, glycerol phosphate dehydrogenase 1, mitochondrial, glycerol-3-phosphate dehydrogenase 2, mitochondrial
Species: mouse

Top Publications

  1. Mizutani S, Gomi H, Hirayama I, Izumi T. Chromosome 2 locus Nidd5 has a potent effect on adiposity in the TSOD mouse. Mamm Genome. 2006;17:375-84 pubmed
    ..The present work physically delineates the major locus for adiposity in the TSOD mouse. ..
  2. DosSantos R, Alfadda A, Eto K, Kadowaki T, Silva J. Evidence for a compensated thermogenic defect in transgenic mice lacking the mitochondrial glycerol-3-phosphate dehydrogenase gene. Endocrinology. 2003;144:5469-79 pubmed
    ..The results support a role for mGPD in thyroid hormone thermogenesis. ..
  3. Brown L, Koza R, Everett C, Reitman M, Marshall L, Fahien L, et al. Normal thyroid thermogenesis but reduced viability and adiposity in mice lacking the mitochondrial glycerol phosphate dehydrogenase. J Biol Chem. 2002;277:32892-8 pubmed
    ..We made a targeted deletion in Gdm1 and produced mice lacking mGPD...
  4. Saheki T, Inoue K, Ono H, Fujimoto Y, Furuie S, Yamamura K, et al. Oral aversion to dietary sugar, ethanol and glycerol correlates with alterations in specific hepatic metabolites in a mouse model of human citrin deficiency. Mol Genet Metab. 2017;120:306-316 pubmed publisher
    ..These findings may underlie the dietary predilections observed in human citrin deficient patients. ..
  5. Saheki T, Inoue K, Ono H, Katsura N, Yokogawa M, Yoshidumi Y, et al. Effects of supplementation on food intake, body weight and hepatic metabolites in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse model of human citrin deficiency. Mol Genet Metab. 2012;107:322-9 pubmed publisher
  6. Saheki T, Iijima M, Li M, Kobayashi K, Horiuchi M, Ushikai M, et al. Citrin/mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency. J Biol Chem. 2007;282:25041-52 pubmed
  7. Ishihara H, Nakazaki M, Kanegae Y, Inukai K, Asano T, Katagiri H, et al. Effect of mitochondrial and/or cytosolic glycerol 3-phosphate dehydrogenase overexpression on glucose-stimulated insulin secretion from MIN6 and HIT cells. Diabetes. 1996;45:1238-44 pubmed
    ..The present data indicate that the emerging hypothesis pointing to mGPDH deficiency as a possible cause of NIDDM needs to be carefully evaluated. ..
  8. Kota V, Rai P, Weitzel J, Middendorff R, Bhande S, Shivaji S. Role of glycerol-3-phosphate dehydrogenase 2 in mouse sperm capacitation. Mol Reprod Dev. 2010;77:773-83 pubmed publisher
    ..Evidence is provided to demonstrate that GPD2 activity is required for ROS generation in mouse spermatozoa during capacitation, failing which, capacitation is impaired. These results imply that GPD2 is involved in sperm capacitation. ..
  9. Chowdhury S, Raha S, Tarnopolsky M, Singh G. Increased expression of mitochondrial glycerophosphate dehydrogenase and antioxidant enzymes in prostate cancer cell lines/cancer. Free Radic Res. 2007;41:1116-24 pubmed
    ..These data suggest that the up-regulation of mGPDH, due to a highly glycolytic environment, contributes to the overall increase in ROS generation and may result in the progression of the cancer. ..

More Information


  1. Anunciado Koza R, Ukropec J, Koza R, Kozak L. Inactivation of UCP1 and the glycerol phosphate cycle synergistically increases energy expenditure to resist diet-induced obesity. J Biol Chem. 2008;283:27688-97 pubmed publisher
    ..Accordingly, a new paradigm for obesity emerges in which the inactivation of major thermogenic pathways force the induction of alternative pathways that increase metabolic inefficiency. ..
  2. Matsutani A, Takeuchi Y, Ishihara H, Kuwano S, Oka Y. Molecular cloning of human mitochondrial glycerophosphate dehydrogenase gene: genomic structure, chromosomal localization, and existence of a pseudogene. Biochem Biophys Res Commun. 1996;223:481-6 pubmed
    ..Genomic cloning also identified a pseudogene located on chromosome 19q13.4. These results provide information useful for analyzing the mGPDH gene in patients with non-insulin dependent diabetes mellitus. ..
  3. Moriyama M, Fujimoto Y, Rikimaru S, Ushikai M, Kuroda E, Kawabe K, et al. Mechanism for increased hepatic glycerol synthesis in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse: Urine glycerol and glycerol 3-phosphate as potential diagnostic markers of human citrin deficiency. Biochim Biophys Acta. 2015;1852:1787-95 pubmed publisher
    ..Therefore, urine G3P and glycerol may represent potential diagnostic markers for human citrin deficiency. ..
  4. Anunciado Koza R, Zhang J, Ukropec J, Bajpeyi S, Koza R, Rogers R, et al. Inactivation of the mitochondrial carrier SLC25A25 (ATP-Mg2+/Pi transporter) reduces physical endurance and metabolic efficiency in mice. J Biol Chem. 2011;286:11659-71 pubmed publisher
    ..However, in the absence of UCP1-based thermogenesis, induction of Slc25a25 in mice with an intact gene may contribute to an alternative thermogenic pathway for the maintenance of body temperature during cold stress. ..
  5. Ravier M, Eto K, Jonkers F, Nenquin M, Kadowaki T, Henquin J. The oscillatory behavior of pancreatic islets from mice with mitochondrial glycerol-3-phosphate dehydrogenase knockout. J Biol Chem. 2000;275:1587-93 pubmed
    ..The oscillatory behavior of beta cells does not depend on the functioning of mGPDH and on metabolic oscillations that would be generated by cyclic activation of this enzyme by Ca(2+). ..
  6. Brown L, Koza R, Marshall L, Kozak L, MacDonald M. Lethal hypoglycemic ketosis and glyceroluria in mice lacking both the mitochondrial and the cytosolic glycerol phosphate dehydrogenases. J Biol Chem. 2002;277:32899-904 pubmed
  7. Mollah M, Ishikawa A. A wild derived quantitative trait locus on mouse chromosome 2 prevents obesity. BMC Genet. 2010;11:84 pubmed publisher
    ..A further understanding of this unique QTL effect at genetic and molecular levels may lead to the discovery of new biological and pathologic pathways associated with obesity. ..
  8. Alfadda A, DosSantos R, Stepanyan Z, Marrif H, Silva J. Mice with deletion of the mitochondrial glycerol-3-phosphate dehydrogenase gene exhibit a thrifty phenotype: effect of gender. Am J Physiol Regul Integr Comp Physiol. 2004;287:R147-56 pubmed
    ..Lower T(4)-to-T(3) conversion in mGPD-/- females and a greater reliance of normal females on mGPD to respond to high-fat diets make the lack of the enzyme more consequential in the female gender. ..
  9. Saheki T, Inoue K, Ono H, Tushima A, Katsura N, Yokogawa M, et al. Metabolomic analysis reveals hepatic metabolite perturbations in citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mice, a model of human citrin deficiency. Mol Genet Metab. 2011;104:492-500 pubmed publisher
    ..Overall, we have identified additional metabolic disturbances in double-KO mice following oral sucrose administration, and provided further evidence for the therapeutic use of sodium pyruvate in our mouse model of citrin deficiency. ..
  10. Eto K, Tsubamoto Y, Terauchi Y, Sugiyama T, Kishimoto T, Takahashi N, et al. Role of NADH shuttle system in glucose-induced activation of mitochondrial metabolism and insulin secretion. Science. 1999;283:981-5 pubmed
    ..The NADH shuttle evidently couples glycolysis with activation of mitochondrial energy metabolism to trigger insulin secretion. ..
  11. Koza R, Kozak U, Brown L, Leiter E, MacDonald M, Kozak L. Sequence and tissue-dependent RNA expression of mouse FAD-linked glycerol-3-phosphate dehydrogenase. Arch Biochem Biophys. 1996;336:97-104 pubmed
    ..between Mus spretus and C57BL/6J were used to map the mouse FAD-linked glycerol-3-phosphate dehydrogenase gene (Gdm1) to chromosome 2, 33 cM from the centromere...
  12. Niedermeyer J, Enenkel B, Park J, Lenter M, Rettig W, Damm K, et al. Mouse fibroblast-activation protein--conserved Fap gene organization and biochemical function as a serine protease. Eur J Biochem. 1998;254:650-4 pubmed
    ..Consistent with the similarity to DPP IV, a chimeric FAP fusion protein expressed in a baculovirus system has dipeptidyl peptidase activity. ..
  13. Mollah M, Ishikawa A. Intersubspecific subcongenic mouse strain analysis reveals closely linked QTLs with opposite effects on body weight. Mamm Genome. 2011;22:282-9 pubmed publisher
    ..These findings illustrate the complex genetic nature of body weight regulation and support the importance of subcongenic mouse analysis to dissect closely linked loci. ..
  14. Barbera A, Gudayol M, Eto K, Corominola H, Maechler P, Miro O, et al. A high carbohydrate diet does not induce hyperglycaemia in a mitochondrial glycerol-3-phosphate dehydrogenase-deficient mouse. Diabetologia. 2003;46:1394-401 pubmed
    ..The phenotype of the mice with an impairment of NADH shuttles does not worsen when fed a high carbohydrate diet; moreover, the diet does not compromise islet function. ..