A-Hydroxyisobutyric acid

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A-Hydroxyisobutyric acid
2D structure for A-Hydroxyisobutyric acid
Chemical Name	2-hydroxy-2-methylpropanoate
Chemical Formula	C₄H₈O₃
CAS Number	594-61-6
Chemical Information	HMDB00729
Biochemical Taxonomy	Hydroxy Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Urine
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 7.0 (0.0-9.0) uM Urine: 0.1 +/- 0.1 umol/mmol creatinine Urine: 0.1+/-0.1 umol/mmol creatinine Urine: 5.5 +/- 2.9 umol/mmol creatinine Urine: 6.2 umol/mmol creatinine Urine: 7.9 +/- 3.7 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Lung Cancer
Other (Monogenic Disorders)	Methacrylic aciduria OMIM: 250620
Abnormal Biofluid Concentrations	Urine (Lung Cancer): 5.0 (0.0-8.0) umol/mmol creatinine
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
Hydroxyisobutyric acid is a metabolite of methyl tert-butyl ether. An elevated level of hydroxyisobutyric acid is identified in urine of patients with isovaleric acidemia (PMID 7133811)