(R)-3-Hydroxyisobutyricacid是l-缬氨酸和胸腺嘧啶途径中的中间产物,在诊断罕见遗传性代谢疾病3-羟异丁酸和甲基丙二醛脱氢酶缺乏症中起着重要的作用。
CAS:1910-47-0
分子式:C4H8O3
分子量:104.1
纯度:98%
存储:Store at -20°C
Background:
(R)-3-Hydroxyisobutyric acid is an intermediate in the pathways of l-valine and thymine and plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria and methylmalonic semialdehyde dehydrogenase deficiency.
[1]. Podebrad F, et al. Stereodifferentiation of 3-hydroxyisobutyric- and 3-aminoisobutyric acid in human urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry. Clin Chim Acta. 2000 Feb 25;292(1-2):93-105.