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Under typical physiological conditions, acetoacetic acid exists as its conjugate base, acetoacetate: AcCH 2 CO 2 H → AcCH 2 CO − 2 + H + Unbound acetoacetate is primarily produced by liver mitochondria from its thioester with coenzyme A (CoA): AcCH 2 C(O)−CoA + OH − → AcCH 2 CO − 2 + H−CoA. The acetoacetate-CoA itself is formed by ...
When 4-maleylacetoacetate isomerase is unable to function properly, the 4-maleylacetoacetate may be converted instead to succinylacetoacetate and further broken down into succinate and acetoacetate by fumarylacetoacetate hydrolase. [3] This image shows the pathway that 4-maleylacetoacetate follows when 4-maleylacetoacetate isomerase is not present.
Ketone bodies are water-soluble molecules or compounds that contain the ketone groups produced from fatty acids by the liver (ketogenesis). [1] [2] Ketone bodies are readily transported into tissues outside the liver, where they are converted into acetyl-CoA (acetyl-Coenzyme A) – which then enters the citric acid cycle (Krebs cycle) and is oxidized for energy.
Ketogenesis pathway. The three ketone bodies (acetoacetate, acetone, and beta-hydroxy-butyrate) are marked within orange boxes. Ketogenesis is the biochemical process through which organisms produce ketone bodies by breaking down fatty acids and ketogenic amino acids.
Acetoacetic ester synthesis is a chemical reaction where ethyl acetoacetate is alkylated at the α-carbon to both carbonyl groups and then converted into a ketone, or more specifically an α-substituted acetone. This is very similar to malonic ester synthesis. Acetoacetic ester synthesis equation
The enzyme acetoacetyl-CoA hydrolase (EC 3.1.2.11) catalyzes the reaction . acetoacetyl-CoA + H 2 O CoA + acetoacetate. This enzyme belongs to the family of hydrolases, specifically those acting on thioester bonds.
Acetoacetyl-CoA synthase (EC 2.3.1.194, NphT7) is an enzyme with systematic name acetyl-CoA:malonyl-CoA C-acetyltransferase (decarboxylating). [1] This enzyme catalyses the following chemical reaction
In enzymology, a butyrate-acetoacetate CoA-transferase (EC 2.8.3.9) is an enzyme that catalyzes the chemical reaction. butanoyl-CoA + acetoacetate butanoate + acetoacetyl-CoA. Thus, the two substrates of this enzyme are butanoyl-CoA and acetoacetate, whereas its two products are butanoate and acetoacetyl-CoA.