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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, ...

Kölker, S. et al., Horswill, A.R. et al., Brämer, C.O. et al., Busch, M. et al., Brämer, C.O. et al., et al.

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Disease relevance of CPD-622

Salmonella enterica serovar Typhimurium LT2 showed increased sensitivity to propionate when the 2-methylcitric acid cycle was blocked [1].
The acnM gene (ORF4) and ORF5, which are required for conversion of 2-methylcitric acid to 2-methylisocitric acid in Ralstonia eutropha HF39, are also located in the prp locus [2].

High impact information on CPD-622

Inhibition of respiratory chain and tricarboxylic acid cycle is most likely induced by synergistically acting alternative metabolites, in particular 2-methylcitric acid, malonic acid, and propionyl-CoA [3].
Salmonella enterica serovar Typhimurium LT2 catabolizes propionate through the 2-methylcitric acid cycle, but the identity of the enzymes catalyzing the conversion of 2-methylcitrate into 2-methylisocitrate is unclear [4].
The phenotype of this double mutant indicates that the aconitase enzymes are required for the 2-methylcitric acid cycle during propionate catabolism [4].
(i) prpC encodes a 2-methylcitric acid synthase (42720 Da) as shown by the measurement of the respective enzyme activity, complementation of a prpC mutant of Salmonella enterica serovar Typhimurium and high sequence similarity [5].
Elevation of 2-methylcitric acid I and II levels in serum, urine, and cerebrospinal fluid of patients with cobalamin deficiency [6].

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].

References

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]
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]
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]
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]
The methylcitric acid pathway in Ralstonia eutropha: new genes identified involved in propionate metabolism. Brämer, C.O., Steinbüchel, A. Microbiology (Reading, Engl.) (2001) [Pubmed]
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]
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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