2-Hydroxybutyric acid

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2-Hydroxybutyric acid
2D structure for 2-Hydroxybutyric acid
Chemical Name	2-hydroxybutanoic acid
Chemical Formula	C₄H₈O₃
CAS Number	600-15-7
Chemical Information	HMDB00008
Biochemical Taxonomy	Hydroxy Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Glycolysis Propanoate Metabolism Pyruvate Metabolism
Biofluid Location	Blood Cerebrospinal Fluid (CSF) Urine
Tissue Location	Liver
Normal Biofluid Concentrations	Blood: 54.0 (8.0-80.0) uM Cerebrospinal Fluid (CSF): 35.0 +/- 24.0 uM Cerebrospinal Fluid (CSF): 37 +/- 21 (10 - 76) uM Cerebrospinal Fluid (CSF): 40 +/- 24 uM Cerebrospinal Fluid (CSF): 85.0 uM Urine: 2.445 (0-4.89) umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Pyruvate dehydrogenase deficiency
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Urine (Pyruvate dehydrogenase deficiency): >25.0 umol/mmol creatinine
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
2-Hydroxybutyric acid is an organic acid that is involved in propanoate metabolism. It is found in urine of patients suffering from lactic acidosis and ketoacidosis. 2-Hydroxybutyric acid generally appears at high concentrations in situations related to deficient energy metabolism (e.g., birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. It was concluded from studies done in the mid 1970's that an increased NADH2/NAD ratio was the most important factor for the production of 2-hydorxybutyric acid (PMID: 168632)

2-Hydroxybutyric acid (or alpha-hydroxybutyrate) is a hydroxybutyric acid with the hydroxyl group on the carbon adjacent to the carboxyl. It is a chiral compound having two enantiomers, D-2-hydroxybutyric acid and L-2-hydroxybutyric acid. 2-Hydroxybutyrate, the conjugate base of 2-hydroxybutyric acid, is produced in mammalian tissues (principaly hepatic) that catabolize L-threonine or synthesize glutathione. Oxidative stress or detoxification demands can dramatically increase the rate of hepatic glutathione synthesis. Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway forming methionine into the transsulfuration pathway forming cystathionine. 2-Hydroxybutyrate is released as a by-product when cystathionine is cleaved to cysteine that is incorporated into glutathione. Chronic shifts in the rate glutathione synthesis may be reflected by urinary excretion of 2-hydroxybutyrate (from Wikipedia).