2-Hydroxy-3-methylbutyric acid

本公司产品仅用于科研实验，不得用于人体或动物的临床与诊断

2-羟基-3-甲基丁酸（2-羟基异戊酸）是苯丙酮尿症、甲基丙二酸血症、丙酸血症、3-酮硫解酶缺乏症、异戊酸血症、3-甲基巴豆酰血糖、3-羟基-3-患者尿液中发现的代谢物。

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.

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2-Hydroxy-3-methylbutanoic acid | (±)-2-Hydroxyisopentanoic Acid | 2-Hydroxyisovaleric Acid

Others

Other Targets

Others

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4026-18-0

118.13

C5H10O3

>98% (HPLC)

DMSO:194.7 mM

CC(C)C(O)C(O)=O

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(-20℃)

With Ice Pack

≥ 2 years