At the beginning of this reaction, 1 molecule of 'Crotonoyl-CoA', and 1 molecule of 'H2O' are present. At the end of this reaction, 1 molecule of '(S)-3-Hydroxybutanoyl-CoA' is present.
This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'enoyl-CoA hydratase hexamer'.
Mitochondrial glutaryl-CoA dehydrogenase (GCDH) catalyzes the reaction of glutaryl-CoA and FAD to form crotonyl-CoA, FADH2, and CO2. The active enzyme is a tetramer of GCDH polypeptides lacking a 44-residue aminoterminal mitochondrial targeting sequence. Mutations in GCDH cause glutaric aciduria type 1 in vivo (Fu et al. 2004; Keyser et al. 2008).
At the beginning of this reaction, 1 molecule of 'FAD', and 1 molecule of 'Butanoyl-CoA' are present. At the end of this reaction, 1 molecule of 'Crotonoyl-CoA', and 1 molecule of 'FADH2' are present.
This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'SCAD acyl-CoA dehydrogenase homotetramer'.
Peroxisomal CROT catalyzes the reaction of 4,8-dimethylnonanoyl-CoA and carnitine to form 4,8-dimethylnonanoylcarnitine and CoASH (Ferdinandusse et al. 1999).