Compound
3-methyl-2-oxovaleric acid
A 2-oxo monocarboxylic acid that is valeric acid carrying oxo- and methyl substituents at C-2 and C-3, respectively. An α-keto acid analogue and metabolite of isoleucine in man, animals and bacteria. Used as a clinical marker for maple syrup urine disease (MSUD).
Definition from ChEBI (CHEBI:35932), read 2026-09-23. CC BY 4.0.
What it touches on the way
Mechanistic reach
7 entities
7 entities reached.
0 reported hops7 assembled from the scaffold
- LDHAProteinUNIPROT:P00338substrate ofInferred only50 paths50 endpoints
- LDHBProteinUNIPROT:P07195substrate ofInferred only23 paths23 endpoints
- BCKDHAProteinUNIPROT:P12694substrate ofInferred only19 paths19 endpoints
- BCKDHBProteinUNIPROT:P21953substrate ofInferred only16 paths16 endpoints
- LDHCProteinUNIPROT:P07864substrate ofInferred only9 paths9 endpoints
- LDHDProteinUNIPROT:Q86WU2substrate ofInferred only5 paths5 endpoints
- LDHAL6BProteinUNIPROT:Q9BYZ2substrate ofInferred only5 paths5 endpoints
What the reference databases state
Statements
9 statements
9 statements · 1 reference database · Subject: 3-methyl-2-oxovaleric acid · Sources: Human-GEM
- L-isoleucine is converted to 3-methyl-2-oxovaleric acidCompoundHuman-GEM MAR03777Inferred only
- L-isoleucine is converted to 3-methyl-2-oxovaleric acidCompoundHuman-GEM MAR03778Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of BCKDHAProteinHuman-GEM MAR20175Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of BCKDHBProteinHuman-GEM MAR20175Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of LDHAProteinHuman-GEM MAR11476Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of LDHAL6BProteinHuman-GEM MAR11476Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of LDHBProteinHuman-GEM MAR11476Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of LDHCProteinHuman-GEM MAR11476Inferred only
- 3-methyl-2-oxovaleric acid is a substrate of LDHDProteinHuman-GEM MAR11476Inferred only
Reactions
8 reactions
2-oxoglutarate(2-)+L-isoleucine3-methyl-2-oxovaleric acid+L-glutamate(1-)
Catalysed by BCAT1
Reaction pageLeft to rightBalancedEC 2.6.1.42GO:0005829
Human-GEM MAR03777CC BY 4.0Reference data
2-oxoglutarate(2-)+L-isoleucine3-methyl-2-oxovaleric acid+L-glutamate(1-)
Catalysed by BCAT2
Reaction pageLeft to rightBalancedEC 2.6.1.42GO:0005759
Human-GEM MAR03778CC BY 4.0Reference data
3-methyl-2-oxovaleric acid+proton3-methyl-2-oxovaleric acid+proton
Reaction pageLeft to rightBalancedGO:0005829, GO:0005759
Human-GEM MAR08093CC BY 4.0Reference data
3-methyl-2-oxovaleric acid3-methyl-2-oxovaleric acid
Reaction pageReversibleBalancedGO:0005615, GO:0005829
Human-GEM MAR09001CC BY 4.0Reference data
Reaction pageReversibleBalance undeterminableGO:0005615
Human-GEM MAR09012CC BY 4.0Reference data
3-methyl-2-oxovaleric acid+proton+NADH(2-)NAD(+)+MAM03176c
Catalysed by LDHB or LDHA or LDHC or LDHD or LDHAL6B
Reaction pageReversibleBalancedEC 1.1.1.27GO:0005829
Human-GEM MAR11476CC BY 4.0Reference data
3-methyl-2-oxovaleric acid+proton+thiamine(1+) diphosphatecarbon dioxide+MAM20079m
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.4GO:0005759
Human-GEM MAR20175CC BY 4.0Reference data
CHEBI:232388+FAD(3-)+hydron3-methyl-2-oxovaleric acid+FADH2(2-)
Catalysed by LDHD
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:82503CC BY 4.0Reference data
Other names
16 names
Resolves to: 3-methyl-2-oxovaleric acid
- Also called
- 2-Oxo-3-methyl-n-valeric acid2-Oxo-3-methylpentanoic acid2-Oxo-3-methylvalerate2-Oxo-3-methylvaleric acid2-Oxoisoleucine2-oxokolavenic acid3-ethyl-3-methylpyruvic acidalpha-keto-beta-methyl-n-valeric acidalpha-keto-beta-methylvaleric acidalpha-oxo-beta-methyl-n-valeric acidalpha-Oxo-beta-methylvaleric acidketoisoleucineα-keto-β-methyl-n-valeric acidα-oxo-β-methyl-n-valeric acidα-oxo-β-methylvaleric acid
- Systematic name
- 3-methyl-2-oxopentanoic acid