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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 ...
In humans, D-β-hydroxybutyrate can be synthesized in the liver via the metabolism of fatty acids (e.g., butyrate), β-hydroxy β-methylbutyrate, and ketogenic amino acids through a series of reactions that metabolize these compounds into acetoacetate, which is the first ketone body that is produced in the fasting state.
Ketone bodies are transported from the liver to other tissues, where acetoacetate and β-hydroxybutyrate can be reconverted to acetyl-CoA to produce reducing equivalents (NADH and FADH 2), via the citric acid cycle. Though it is the source of ketone bodies, the liver cannot use them for energy because it lacks the enzyme thiophorase (β ...
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.
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 ...
Two acetyl-CoA molecules condense to form acetoacetyl-CoA, which gives rise to the formation of acetoacetate and β-hydroxybutyrate. [16] Acetoacetate, β-hydroxybutyrate, and their spontaneous breakdown product acetone [ 18 ] are frequently, but confusingly, known as ketone bodies (as they are not "bodies" at all, but water-soluble chemical ...
Acetoacetate is an intermediate in the Krebs cycle which releases energy from sugars and carbohydrates. [22] In medicine, acetone, acetoacetate, and beta-hydroxybutyrate are collectively called ketone bodies, generated from carbohydrates, fatty acids, and amino acids in most vertebrates, including humans.
3-Hydroxy-3-methylglutaryl-CoA lyase (or HMG-CoA lyase) is an enzyme (EC 4.1.3.4 that in human is encoded by the HMGCL gene located on chromosome 1. It is a key enzyme in ketogenesis (ketone body formation). It is a ketogenic enzyme in the liver that catalyzes the formation of acetoacetate from HMG-CoA within the mitochondria.