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Beta-hydroxybutyrate, acetoacetate, and acetone collectively are called ketone bodies. The first two are synthesized from acetyl-CoA, in the mitochondria of liver cells; acetone is formed by spontaneous decarboxylation of acetoacetate. Ketone body synthesis in liver is effectively irreversible because the enzyme that catalyzes the conversion of acetoacetate to acetoacetyl-CoA is not present in liver cells. Ketone bodies, unlike fatty acids and triglycerides, are water-soluble. They are exported from the liver, and are taken up by other tissues, notably brain and skeletal and cardiac muscle. There, they are broken down to acetyl-CoA which is oxidized via the TCA cycle to yield energy. In healthy individuals, this pathway of ketone body synthesis and utilization is most active during extended periods of fasting. Copyright Text is available under the Creative Commons Attribution/Share-Alike License. See for details. Source: Wikipedia, Beta hydroxybutyrate (Wikipedia)

Increases in beta-hydroxybutyrate are due to excessive fatty acid oxidation. Elevated levels are seen in gluconeogenesis, PHD complex deficiency, respiratory chain defects, IVA, PA, MMA, multiple carboxylase deficiency, 3-methylcrotonyl-CoA carboxylase deficiency, glyceroluria, MSUD, GA I, MAD deficiency, Beta-ketothiolase deficiencies, 2-amino/2-ketoadipic acidemia,mitochondrial SCHAD,and fatty acids oxidation deficiency. (Kumps A, Duez P, Mardens Y. Metabolic, Nutritional, Iatrogenic, and Artifactual Sources of Urinary Organic Acids: A Comprehensive Table. Clinical Chemistry 2002, 48:708-717.) Source: Regenstrief Institute

Basic Part Properties

Part Display Name
Beta hydroxybutyrate
Part Type
Component (Describes the core component or analyte measured)
Created On
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Language Variants Get Info

Tag Language Translation
zh-CN Chinese (China) Beta 羟基丁酸
Synonyms: 3-羟基丁酸;Beta-羟基丁酸;BOHB;B-OH-丁酸;β羟基丁酸;β-羟基丁酸
fr-CA French (Canada) Bêta-hydroxybutyrate
et-EE Estonian (Estonia) Beetahüdroksübutüraat
es-ES Spanish (Spain) Beta Hidroxibutirato
it-IT Italian (Italy) Beta idrossibutirrato
tr-TR Turkish (Turkey) Beta Hidroksibütirat
ru-RU Russian (Russian Federation) Бета гидроксибутират
nl-NL Dutch (Netherlands) bèta-hydroxybutyraat
fr-BE French (Belgium) Béta-hydroxybutyrate