A valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position; an alpha-hydroxy analogue of leucine. A bacterial metabolite, it has also been isolated from amniotic fluid, was found in a patient with dihydrolipoyl dehydrogenase deficiency and is present in the urine of patients with short bowel syndrome.
ChEBI ID: 59783
There are 2 compounds belonging to this class, involving 0 studies.
| Member | Definition | Role |
|---|---|---|
| (S)-2-hydroxy-4-methylpentanoic-acid | The (S)-enantiomer of 2-hydroxy-4-methylpentanoic acid. Derived from the metabolism of the branched-chain amino acids, it belongs to the 2-hydroxycarboxylic acid group of amino acid metabolites. | |
| (R)-2-hydroxy-4-methylpentanoic-acid | The (R)-enantiomer of 2-hydroxy-4-methylpentanoic acid. Found in patients with short-bowel syndrome (an inborn error of metabolism), and in maple syrup urine disease, MSUD. |
| Pre-1990 | 1990-2000 | 2001-2010 | 2011-2020 | Post-2020 |
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