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(R)-3-Hydroxyisobutyric acid
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
(R)-3-Hydroxyisobutyric acid图片
CAS NO:1910-47-0
包装:5mg
规格:98%
市场价:1337元
分子量:104.1

产品介绍
(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.