Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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Chemical Compound Review:

ACMC-1AFVL 2-hydroxy-3-methyl-butanoic acid

Synonyms: AG-F-42618, CHEMBL1162479, ACMC-209e7s, CHEBI:60645, HMDB00407, ...

Landaas, S. et al., Jakobs, C. et al.

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Disease relevance of NSC 227884

The occurrence of 2-hydroxyisovaleric acid in patients with lactic acidosis and ketoacidosis [1].
The concentration of 2-hydroxyisovaleric acid was not useful in the prenatal diagnosis of a fetus with maple syrup urine disease [2].

Analytical, diagnostic and therapeutic context of NSC 227884

Examination of urine samples by capillary column gas chromatography and mass spectrometry showed the presence of 2-hydroxyisovaleric acid together with trace amounts of the three branched-chain 2-keto acids in five patients with lactic and ketoacidosis [1].

References

The occurrence of 2-hydroxyisovaleric acid in patients with lactic acidosis and ketoacidosis. Landaas, S., Jakobs, C. Clin. Chim. Acta (1977) [Pubmed]
Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Jakobs, C., Sweetman, L., Nyhan, W.L. Clin. Chim. Acta (1984) [Pubmed]

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