35) The MVA pathway begins with the synthesis of acetoacetyl-CoA, which was first reported to be biosynthesized via a thioester-dependent Claisen condensation reaction between two molecules of acetyl-CoA, and is catalyzed by acetoacetyl-CoA thiolase
Activation of liver carnitine palmitoyltransferase-1 and mitochondrial acetoacetyl-CoA thiolase
is associated with elevated ketone body levels in the elasmobranch Squalus acanthias.
The second genetic disorder included in this study is an organic acid disorder called Mitochondrial acetoacetyl-CoA thiolase
(T2), commonly known as Beta-ketothiolase deficiency.
Activities of 3-hydroxybutyrate dehydrogenase, 3-oxoacid CoA-transferase and acetoacetyl-CoA thiolase
in relation to ketone-body utilisation in muscles from vertebrates and invertebrates.