2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria, 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 I, galactosemia, and maple syrup urine disease.
结构分类
Ketones, Aldehydes, Acids
参考文献
[1]. Pan YM, et al. Improved procedure for the analysis of gamma-hydroxybutyrate and ethylene glycol in whole blood. J Anal Toxicol. 2001 Jul-Aug;25(5):328-32.
[2].Magni F, et al. Simultaneous determination of plasma levels of alpha-ketoisocaproic acid and leucine and evaluation of alpha-[1-13C]ketoisocaproic acid and [1-13C]leucine enrichment by gas chromatography-mass spectrometry. Anal Biochem. 1994 Aug 1;220(2):308-14.
溶解度数据
DMSO : 100 mg/mL (846.53 mM; Need ultrasonic)
DMSO: 194.7 mM( < 1 mg/mL refers to the product slightly soluble or insoluble )