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3-methyl-2-oxobutanoate

362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API

Inferred

Every route here is a hypothesis drawn from reference reactions (Rhea and Human-GEM), not a finding. Currency species such as water, ATP and NAD(H) never form a step. Evidence key

7 compounds within 3 steps: 1 in 1, 1 in 2, 5 in 3

Does it reach a compound?

One step

2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
1. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)
Catalysed by BCKDHA, BCKDHBmitochondrial matrix

Two steps

thiamine(1+) diphosphateReach from here →
1. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)
2. lipoamide to S-(2-methylpropanoyl)... conversion
Catalysed by BCKDHA, BCKDHB, DBTmitochondrial matrix

Three steps

CHEBI:16284Reach from here →
Catalysed by BCKDHA, BCKDHB, DBT, SLC25A19mitochondrial matrix, cytosolOne of 2 routes
2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →

Catalysed by BCKDHA, BCKDHB, DBTmitochondrial matrix

3-methyl-1-hydroxybutylthiamine diphosphateReach from here →

branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)
lipoamide to S-(2-methylpropanoyl)... conversion
branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

Catalysed by BCKDHA, BCKDHB, DBTmitochondrial matrix

thiamine triphosphateReach from here →

branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)
lipoamide to S-(2-methylpropanoyl)... conversion
ATP:thiamine-diphosphate phosphotransferase

Catalysed by BCKDHA, BCKDHB, DBT, TPK1mitochondrial matrix, cytosol

2-(1-hydroxyethyl)thiamine diphosphateReach from here →

branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)
lipoamide to S-(2-methylpropanoyl)... conversion
pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

Catalysed by BCKDHA, BCKDHB, DBT, PDHA1 and 2 moremitochondrial matrix