Acyl-CoA dehydrogenases (BCAA branch)
Cofactors used
Clinical / pharmacological. MCAD deficiency: hypoketotic hypoglycaemia, dicarboxylic aciduria, SIDS-like presentation
What it does, reaction by reaction
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion
Isoleucine-derived 2-methylbutyryl-CoA → tiglyl-CoA → 2-methyl-3-hydroxybutyryl-CoA → 2-methylacetoacetyl-CoA → acetyl-CoA + propionyl-CoA
Converts Isoleucine-derived 2-methylbutyryl-CoA into Tiglyl-CoA
› Notes
Dehydrogenase, hydratase, dehydrogenase, and thiolase reactions process the acyl chain; the first dehydrogenation uses FAD and the hydroxyacyl dehydrogenase uses NAD⁺. Propionyl-CoA is converted by propionyl-CoA carboxylase (biotin, ATP, bicarbonate), methylmalonyl-CoA epimerase, and methylmalonyl-CoA mutase (adenosylcobalamin) to succinyl-CoA, so isoleucine is both ketogenic and glucogenic.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
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