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Chemical Compound Review

zlchem 1259     3-hydroxypentanedioic acid

Synonyms: CHEMBL556937, AG-G-37715, CHEBI:39980, HMDB00428, ZLE0034, ...
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Disease relevance of 3-hydroxypentanedioic acid


High impact information on 3-hydroxypentanedioic acid


Biological context of 3-hydroxypentanedioic acid


Anatomical context of 3-hydroxypentanedioic acid


Associations of 3-hydroxypentanedioic acid with other chemical compounds


Gene context of 3-hydroxypentanedioic acid


Analytical, diagnostic and therapeutic context of 3-hydroxypentanedioic acid


  1. Novel metabolic and molecular findings in hepatic carnitine palmitoyltransferase I deficiency. Korman, S.H., Waterham, H.R., Gutman, A., Jakobs, C., Wanders, R.J. Mol. Genet. Metab. (2005) [Pubmed]
  2. IGF-1 and bFGF reduce glutaric acid and 3-hydroxyglutaric acid toxicity in striatal cultures. Bjugstad, K.B., Zawada, W.M., Goodman, S., Free, C.R. J. Inherit. Metab. Dis. (2001) [Pubmed]
  3. Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation. Molven, A., Matre, G.E., Duran, M., Wanders, R.J., Rishaug, U., Njølstad, P.R., Jellum, E., Søvik, O. Diabetes (2004) [Pubmed]
  4. 3-Hydroxyglutaric acid fails to affect the viability of primary neuronal rat cells. Freudenberg, F., Lukacs, Z., Ullrich, K. Neurobiol. Dis. (2004) [Pubmed]
  5. Endothelial effects of 3-hydroxyglutaric acid: implications for glutaric aciduria type I. Mühlhausen, C., Ott, N., Chalajour, F., Tilki, D., Freudenberg, F., Shahhossini, M., Thiem, J., Ullrich, K., Braulke, T., Ergün, S. Pediatr. Res. (2006) [Pubmed]
  6. 3-Hydroxyglutaric acid moderately impairs energy metabolism in brain of young rats. Latini, A., Rodriguez, M., Borba Rosa, R., Scussiato, K., Leipnitz, G., Reis de Assis, D., da Costa Ferreira, G., Funchal, C., Jacques-Silva, M.C., Buzin, L., Giugliani, R., Cassina, A., Radi, R., Wajner, M. Neuroscience (2005) [Pubmed]
  7. Maturation-dependent neurotoxicity of 3-hydroxyglutaric and glutaric acids in vitro: a new pathophysiologic approach to glutaryl-CoA dehydrogenase deficiency. Kölker, S., Ahlemeyer, B., Krieglstein, J., Hoffmann, G.F. Pediatr. Res. (2000) [Pubmed]
  8. 3-Hydroxyglutaric acid induces oxidative stress and decreases the antioxidant defenses in cerebral cortex of young rats. Latini, A., Borba Rosa, R., Scussiato, K., Llesuy, S., Belló-Klein, A., Wajner, M. Brain Res. (2002) [Pubmed]
  9. Quinolinic acid reduces the antioxidant defenses in cerebral cortex of young rats. Leipnitz, G., Schumacher, C., Scussiato, K., Dalcin, K.B., Wannmacher, C.M., Wyse, A.T., Dutra-Filho, C.S., Wajner, M., Latini, A. Int. J. Dev. Neurosci. (2005) [Pubmed]
  10. Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen. Kölker, S., Hoffmann, G.F., Schor, D.S., Feyh, P., Wagner, L., Jeffrey, I., Pourfarzam, M., Okun, J.G., Zschocke, J., Baric, I., Bain, M.D., Jakobs, C., Chalmers, R.A. Neuropediatrics. (2003) [Pubmed]
  11. Sensitivity and specificity of free and total glutaric acid and 3-hydroxyglutaric acid measurements by stable-isotope dilution assays for the diagnosis of glutaric aciduria type I. Baric, I., Wagner, L., Feyh, P., Liesert, M., Buckel, W., Hoffmann, G.F. J. Inherit. Metab. Dis. (1999) [Pubmed]
  12. Nerve cell lesions caused by 3-hydroxyglutaric acid: a possible mechanism for neurodegeneration in glutaric acidaemia I. Flott-Rahmel, B., Falter, C., Schluff, P., Fingerhut, R., Christensen, E., Jakobs, C., Musshoff, U., Fautek, J.D., Deufel, T., Ludolph, A., Ullrich, K. J. Inherit. Metab. Dis. (1997) [Pubmed]
  13. Quantification of 3-hydroxyglutaric acid in urine, plasma, cerebrospinal fluid and amniotic fluid by stable-isotope dilution negative chemical ionization gas chromatography-mass spectrometry. Schor, D.S., Verhoeven, N.M., Struys, E.A., ten Brink, H.J., Jakobs, C. J. Chromatogr. B Analyt. Technol. Biomed. Life Sci. (2002) [Pubmed]
  14. Glutaric aciduria type I: pathomechanisms of neurodegeneration. Ullrich, K., Flott-Rahmel, B., Schluff, P., Musshoff, U., Das, A., Lücke, T., Steinfeld, R., Christensen, E., Jakobs, C., Ludolph, A., Neu, A., Röper, R. J. Inherit. Metab. Dis. (1999) [Pubmed]
  15. Modulation of glutamatergic and GABAergic neurotransmission in glutaryl-CoA dehydrogenase deficiency. Wajner, M., Kölker, S., Souza, D.O., Hoffmann, G.F., de Mello, C.F. J. Inherit. Metab. Dis. (2004) [Pubmed]
  16. Glutaryl-CoA dehydrogenase deficiency presenting as 3-hydroxyglutaric aciduria. Nyhan, W.L., Zschocke, J., Hoffmann, G., Stein, D.E., Bao, L., Goodman, S. Mol. Genet. Metab. (1999) [Pubmed]
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