A-Ketoisovaleric acid

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A-Ketoisovaleric acid
2D structure for A-Ketoisovaleric acid
Chemical Name	3-methyl-2-oxo-butanoic acid
Chemical Formula	C₅H₈O₃
CAS Number	759-05-7
Chemical Information	HMDB00019
Biochemical Taxonomy	Keto-Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Pantothenate and CoA Biosynthesis Valine, Leucine and Isoleucine Biosynthesis Valine, Leucine and Isoleucine Degradation
Biofluid Location	Blood Cerebrospinal Fluid (CSF) Urine
Tissue Location	Most Tissues
Normal Biofluid Concentrations	Blood: 11.0 +/- 1.7 uM Blood: 14.0 (0.0-28.0) uM Cerebrospinal Fluid (CSF): 6 +/- 3 uM Cerebrospinal Fluid (CSF): 8.2 +/- 6.8 uM Urine: 0.07 (0.01-0.45) umol/mmol creatinine Urine: 0.13 (0.00-0.54) 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
A branched chain organic acid which is a precursor to leucine and valine synthesis. It is also a degradation product from valine. The enzyme dihydroxy-acid dehydratase catalyzes the fourth step in the biosynthesis of isoleucine and valine, through the dehydration of 2, 3-dihydroxy-isovaleic acid into alpha-ketoisovaleric acid.