2-Ketobutyric acid

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2-Ketobutyric acid
2D structure for 2-Ketobutyric acid
Chemical Name	2-oxobutanoic acid
Chemical Formula	C₄H₆O₃
CAS Number	600-18-0
Chemical Information	HMDB00005
Biochemical Taxonomy	Keto-Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Butanoate Metabolism Glycolysis Methionine Metabolism Pantothenate and CoA Biosynthesis Propanoate Metabolism Pyruvate Metabolism Valine, Leucine and Isoleucine Biosynthesis
Biofluid Location	Blood Cerebrospinal Fluid (CSF) Urine
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 3.33 +/- 5.00 uM Blood: 8.6 (6.0-11.2) uM Blood: 9.4 (3.4-15.4) uM Cerebrospinal Fluid (CSF): 4.8 (3.4-6.2) uM Urine: 0.39 (0.05-2.2) umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
2-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted to propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted to succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.