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Chemical Compound Review

AGN-PC-00JZS5     2-hydroxybutane-1,2,3- tricarboxylic acid

Synonyms: AG-G-20063, CHEBI:30835, HMDB00379, AC1Q5SNI, CTK2F3922, ...
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Disease relevance of CPD-622


High impact information on CPD-622


Anatomical context of CPD-622

  • BACKGROUND: Despite some clinical applications of 2-methylcitric acid (2-MCA) determination in urine and amniotic fluid, a diagnostic use of 2-MCA levels in serum is not common practice [7].


  1. Studies of propionate toxicity in Salmonella enterica identify 2-methylcitrate as a potent inhibitor of cell growth. Horswill, A.R., Dudding, A.R., Escalante-Semerena, J.C. J. Biol. Chem. (2001) [Pubmed]
  2. Identification of the 2-methylcitrate pathway involved in the catabolism of propionate in the polyhydroxyalkanoate-producing strain Burkholderia sacchari IPT101(T) and analysis of a mutant accumulating a copolyester with higher 3-hydroxyvalerate content. Brämer, C.O., Silva, L.F., Gomez, J.G., Priefert, H., Steinbüchel, A. Appl. Environ. Microbiol. (2002) [Pubmed]
  3. Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain. Kölker, S., Schwab, M., Hörster, F., Sauer, S., Hinz, A., Wolf, N.I., Mayatepek, E., Hoffmann, G.F., Smeitink, J.A., Okun, J.G. J. Biol. Chem. (2003) [Pubmed]
  4. In vitro conversion of propionate to pyruvate by Salmonella enterica enzymes: 2-methylcitrate dehydratase (PrpD) and aconitase Enzymes catalyze the conversion of 2-methylcitrate to 2-methylisocitrate. Horswill, A.R., Escalante-Semerena, J.C. Biochemistry (2001) [Pubmed]
  5. The methylcitric acid pathway in Ralstonia eutropha: new genes identified involved in propionate metabolism. Brämer, C.O., Steinbüchel, A. Microbiology (Reading, Engl.) (2001) [Pubmed]
  6. Elevation of 2-methylcitric acid I and II levels in serum, urine, and cerebrospinal fluid of patients with cobalamin deficiency. Allen, R.H., Stabler, S.P., Savage, D.G., Lindenbaum, J. Metab. Clin. Exp. (1993) [Pubmed]
  7. Validated capillary gas chromatographic-mass spectrometric assay to determine 2-methylcitric acid I and II levels in human serum by using a pulsed splitless injection procedure. Busch, M., Stein, G., Poppitz, W., Hein, G., Müller, A. J. Chromatogr. B Analyt. Technol. Biomed. Life Sci. (2002) [Pubmed]
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