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Both acetoacetate and beta-hydroxybutyrate are acidic, and, if levels of these ketone bodies are too high, the pH of the blood drops, resulting in ketoacidosis. Ketoacidosis is known to occur in untreated type I diabetes (see diabetic ketoacidosis ) and in alcoholics after prolonged binge-drinking without intake of sufficient carbohydrates (see ...
Acetoacetate, beta-hydroxybutyrate, and their spontaneous breakdown product, acetone, [9] are known as ketone bodies. The ketone bodies are released by the liver into the blood. All cells with mitochondria can take ketone bodies up from the blood and reconvert them into acetyl-CoA, which can then be used as fuel in their citric acid cycles, as ...
β-Hydroxybutyrate, BHB, is also synthesized within liver cells; this is accomplished through the metabolism of fatty acids. Through a series of reactions, acetoacetate is first produced; and it is this acetoacetate that is reduced into β-hydroxybutyrate, catalyzed by the β-hydroxybutyrate dehydrogenase enzyme.
A pH measurement is performed to detect acidosis. Blood from a vein is adequate, as there is little difference between the arterial and the venous pH; arterial samples are only required if there are concerns about oxygen levels. [6] Ketones can be measured in the urine (acetoacetate) and blood (β-hydroxybutyrate).
In whole blood (g/cm 3) In plasma or serum (g/cm 3) Water: Solvent 0.81-0.86 0.93-0.95 Acetoacetate: Produced in liver 8-40 × 10 −7: 4-43 × 10 −7: Acetone: product of bodyfat breakdown 3-20 × 10 −6: Acetylcholine: Neurotransmitter of the parasympathetic nervous system: 6.6-8.2 × 10 −8: Adenosine triphosphate: Energy storage total 3. ...
n/a n/a Ensembl n/a n/a UniProt n a n/a RefSeq (mRNA) n/a n/a RefSeq (protein) n/a n/a Location (UCSC) n/a n/a PubMed search n/a n/a Wikidata View/Edit Human Hydroxymethylglutaryl-CoA lyase HMG-CoA lyase dimer, Human Identifiers EC no. 4.1.3.4 CAS no. 9030-83-5 Databases IntEnz IntEnz view BRENDA BRENDA entry ExPASy NiceZyme view KEGG KEGG entry MetaCyc metabolic pathway PRIAM profile PDB ...
Alternatively, acetoacetate can spontaneously degrade to a third ketone body (acetone) and carbon dioxide, which generates much greater concentrations of acetoacetate and D-β-hydroxybutyrate. The resulting ketone bodies cannot be used for energy by the liver so are exported from the liver to supply energy to the brain and peripheral tissues.
Under these conditions, acetyl-CoA is diverted to the formation of acetoacetate and beta-hydroxybutyrate. [10] Acetoacetate, beta-hydroxybutyrate, and their spontaneous breakdown product, acetone, are frequently, but confusingly, known as ketone bodies (as they are not "bodies" at all, but water-soluble chemical substances). The ketones are ...