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Chemical Compound Review

ACMC-1AQEZ     2-hydroxypentanedioic acid

Synonyms: AG-D-62814, CHEBI:17084, AC1Q5SSV, CTK0B4621, AR-1E2744, ...
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Disease relevance of hydroxyglutarate


High impact information on hydroxyglutarate


Chemical compound and disease context of hydroxyglutarate


Biological context of hydroxyglutarate


Associations of hydroxyglutarate with other chemical compounds


Gene context of hydroxyglutarate

  • We determined the optical isomer of the 2-hydroxyglutaric acid (2HG) that was elevated in the urine of five Japanese children with a mild form of glutaric aciduria type II (GA2), caused by a deficiency of electron transfer flavoprotein (ETF) or ETF-ubiquinone oxidoreductase (ETF-QO) [16].
  • We propose that in the hydroxyglutarate pathway these oxidoreductases work in the reverse direction, whereby the reduction of ferredoxin by NADH is driven by the Na(+) gradient [17].

Analytical, diagnostic and therapeutic context of hydroxyglutarate


  1. Re-face stereospecificity at C4 of NAD(P) for alcohol dehydrogenase from Methanogenium organophilum and for (R)-2-hydroxyglutarate dehydrogenase from Acidaminococcus fermentans as determined by 1H-NMR spectroscopy. Berk, H., Buckel, W., Thauer, R.K., Frey, P.A. FEBS Lett. (1996) [Pubmed]
  2. On the dehydration of (R)-lactate in the fermentation of alanine to propionate by Clostridium propionicum. Schweiger, G., Buckel, W. FEBS Lett. (1984) [Pubmed]
  3. Pleomorphism of fusobacteria isolated from the cockroach hindgut. Foglesong, M.A., Cruden, D.L., Markovetz, A.J. J. Bacteriol. (1984) [Pubmed]
  4. Analysis of the fermentation pathways of clostridia using double labelled glutamade. Buckel, W. Arch. Microbiol. (1980) [Pubmed]
  5. A sodium ion gradient as energy source for Peptostreptococcus asaccharolyticus. Wohlfarth, G., Buckel, W. Arch. Microbiol. (1985) [Pubmed]
  6. 5-Oxo-L-prolinase (L-pyroglutamate hydrolase). Studies of the chemical mechanism. Griffith, O.W., Meister, A. J. Biol. Chem. (1981) [Pubmed]
  7. The sodium ion translocating glutaconyl-CoA decarboxylase from Acidaminococcus fermentans: cloning and function of the genes forming a second operon. Braune, A., Bendrat, K., Rospert, S., Buckel, W. Mol. Microbiol. (1999) [Pubmed]
  8. 7-Methylpterin and 7-methyllumizine: oxidative degradation products of 7-methyl-substituted pteridines in methanogenic bacteria. White, R.H. J. Bacteriol. (1985) [Pubmed]
  9. L-Histidinol phosphate aminotransferase from Salmonella typhimurium. Kinetic behavior and sequence at the pyridoxal-P binding site. Hsu, L.C., Okamoto, M., Snell, E.E. Biochimie (1989) [Pubmed]
  10. Equilibrium constants of several reactions involved in the fermentation of glutamate. Buckel, W., Miller, S.L. Eur. J. Biochem. (1987) [Pubmed]
  11. The reversible dehydration of (R)-2-hydroxyglutarate to (E)-glutaconate. Buckel, W. Eur. J. Biochem. (1980) [Pubmed]
  12. Purification of 2-hydroxyglutaryl-CoA dehydratase from Acidaminococcus fermentans. An iron-sulfur protein. Schweiger, G., Dutscho, R., Buckel, W. Eur. J. Biochem. (1987) [Pubmed]
  13. Glutaconate CoA-transferase from Acidaminococcus fermentans. Buckel, W., Dorn, U., Semmler, R. Eur. J. Biochem. (1981) [Pubmed]
  14. Determination of the absolute configuration of 2-hydroxyglutaric acid and 5-oxoproline in urine samples by high-resolution NMR spectroscopy in the presence of chiral lanthanide complexes. Bal, D., Gradowska, W., Gryff-Keller, A. Journal of pharmaceutical and biomedical analysis. (2002) [Pubmed]
  15. Structural basis for stereo-specific catalysis in NAD(+)-dependent (R)-2-hydroxyglutarate dehydrogenase from Acidaminococcus fermentans. Martins, B.M., Macedo-Ribeiro, S., Bresser, J., Buckel, W., Messerschmidt, A. FEBS J. (2005) [Pubmed]
  16. Identification of the D-enantiomer of 2-hydroxyglutaric acid in glutaric aciduria type II. Watanabe, H., Yamaguchi, S., Saiki, K., Shimizu, N., Fukao, T., Kondo, N., Orii, T. Clin. Chim. Acta (1995) [Pubmed]
  17. Sodium ion pumps and hydrogen production in glutamate fermenting anaerobic bacteria. Boiangiu, C.D., Jayamani, E., Brugel, D., Herrmann, G., Kim, J., Forzi, L., Hedderich, R., Vgenopoulou, I., Pierik, A.J., Steuber, J., Buckel, W. J. Mol. Microbiol. Biotechnol. (2005) [Pubmed]
  18. Measurement of urinary D- and L-2-hydroxyglutarate enantiomers by stable-isotope-dilution liquid chromatography-tandem mass spectrometry after derivatization with diacetyl-L-tartaric anhydride. Struys, E.A., Jansen, E.E., Verhoeven, N.M., Jakobs, C. Clin. Chem. (2004) [Pubmed]
  19. L-2-Hydroxyglutaric aciduria: an inborn error of metabolism? Duran, M., Kamerling, J.P., Bakker, H.D., van Gennip, A.H., Wadman, S.K. J. Inherit. Metab. Dis. (1980) [Pubmed]
  20. Chiral liquid chromatography tandem mass spectrometry in the determination of the configuration of 2-hydroxyglutaric acid in urine. Rashed, M.S., AlAmoudi, M., Aboul-Enein, H.Y. Biomed. Chromatogr. (2000) [Pubmed]
  21. L-2 hydroxyglutaric aciduria: proton magnetic resonance spectroscopy and diffusion magnetic resonance imaging findings. Sener, R.N. Journal of computer assisted tomography. (2003) [Pubmed]
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