Compound

2-hydroxy-4-methylvaleric acid

A valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position; an α-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.

Definition from ChEBI (CHEBI:59783), read 2026-09-23. CC BY 4.0.

CHEBI:59783ChEBI

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Structure

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A generated conformer of 2-hydroxy-4-methylvaleric acid — one low-energy arrangement PubChem computed, not a measured structure. Hydrogens are not drawn; the formula counts them.

Class: Organic compound
Formula: C6H12O3
Mass: 132.159 g/mol
Charge: 0
InChIKey: LVRFTAZAXQPQHI-UHFFFAOYSA-N
SMILES: CC(C)CC(O)C(=O)O
Look it up: ChEBI PubChem By InChIKey

Identity from ChEBI, geometry from PubChem. Not evidence.

What it touches on the way

Mechanistic reach

10 entities

10 entities reached.

0 reported hops10 assembled from the scaffold

What the reference databases state

Statements

20 statements

20 statements · 1 reference database · Subject: 2-hydroxy-4-methylvaleric acid · Sources: Human-GEM

Order

4-methyl-2-oxopentanoate is converted to 2-hydroxy-4-methylvaleric acidCompoundHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is converted to 4-methyl-2-oxopentanoateCompoundHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is metabolised by ADH5ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of AKR1B10ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of AKR1B15ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of AKR1C1ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of BDH2ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of CBR4ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of CTBP1ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of DHRS2ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of HSD11B1LProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of HSD17B11ProteinHuman-GEM MAR06925Inferred only

Show the remaining 8

2-hydroxy-4-methylvaleric acid is a substrate of HSD17B8ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of HTATIP2ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of MDH1BProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of SDR16C5ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of SDR42E1ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of SDR42E2ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of SDR9C7ProteinHuman-GEM MAR06925Inferred only
2-hydroxy-4-methylvaleric acid is a substrate of WWOXProteinHuman-GEM MAR06925Inferred only

Reactions

2 reactions

4-methyl-2-oxopentanoate+proton+NADH(2-)2-hydroxy-4-methylvaleric acid+NAD(+)
Catalysed by DHRS2 or HTATIP2 or MDH1B or CBR4 or CTBP1 or BDH2 or HSD11B1L or SDR9C7 or SDR16C5 or SDR42E2 or SDR42E1 or WWOX and 6 more alternatives
Reaction pageReversibleBalancedEC 1.1.1.-GO:0005829
Human-GEM MAR06925CC BY 4.0Reference data
2-hydroxy-4-methylvaleric acid+dioxygen4-methyl-2-oxopentanoate+hydrogen peroxide
Reaction pageDirection not assertedBalance undeterminable
Rhea RHEA:69380CC BY 4.0Reference data

Other names

8 names

Resolves to: 2-hydroxy-4-methylvaleric acid

Also called: 2-hydroxy-4-methyl-pentanoic acid2-hydroxyisocaproic acid2-hydroxyisohexanoic acidalpha-Hydroxyisocaproic acidFA 6:0;OLeucic acidleucinic acid
Systematic name: 2-hydroxy-4-methylpentanoic acid