Reaction: alpha-methyl-acetoacetyl-CoA + CoA => propionyl-CoA + acetyl-CoA
- in pathway: Branched-chain amino acid catabolism
Mitochondrial acetyl-CoA acetyltransferase (ACAT1) catalyzes the reaction of alpha-methyl-acetoacetyl-CoA and CoA to form propionyl-CoA and acetyl-CoA. Structural studies have shown the active enzyme to be a tetramer of ACAT1 polypeptides whose aminoterminal 34 residues, a mitochondrial targeting sequence, have been removed (Haapalainen et al. 2007).
Reaction - small molecule participants:
PROP-CoA [mitochondrial matrix]
Ac-CoA [mitochondrial matrix]
CoA-SH [mitochondrial matrix]
2MACA-CoA [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70844
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Reaction input - small molecules:
coenzyme A(4-)
2-methylacetoacetyl-CoA
Reaction output - small molecules:
propionyl-CoA
acetyl-CoA(4-)
Reactome.org link: R-HSA-70844