2-Hydroxy-3-methylbutyric acid

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2-Hydroxy-3-methylbutyric acid
2D structure for 2-Hydroxy-3-methylbutyric acid
Chemical Name	(2S)-2-hydroxy-3-methyl-butanoic acid
Chemical Formula	C₅H₁₀O₃
CAS Number	4026-18-0
Chemical Information	HMDB00407
Biochemical Taxonomy	Hydroxy Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Cerebrospinal Fluid (CSF)
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 4.5 (2.9-6.2) uM Blood: 7.7 (0.0-19.0) uM Cerebrospinal Fluid (CSF): 6.8 +/- 6.8 uM Cerebrospinal Fluid (CSF): 8 +/- 6 uM
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-Hydroxy-3-methylbutyric acid is a metabolite found in the urine of patients with Phenylketonuria (PMID 7978272), Methylmalonic acidemia, Propionic acidemia, 3-Ketothiolase deficiency, Isovaleric acidemia, 3-Methylcrotonylglycemia, 3-Hydroxy-3-methylglutaric acidemia, Multiple carboxylase deficiency, Glutaric aciduria, Ornithine transcarbamylase deficiency, glyceroluria, Tyrosinemia type 1, Galactosemia, and Maple syrup urine disease (PMID 11048741)