(R)-3-Hydroxyisobutyric acid |
| カタログ番号GC30661 |
(R)-3-ヒドロキシイソ酪酸は、l-バリンとチミンの経路の中間体であり、非常にまれな遺伝性代謝疾患である 3-ヒドロキシイソ酪酸尿症とメチルマロン酸セミアルデヒド脱水素酵素欠損症の診断に重要な役割を果たします。
Products are for research use only. Not for human use. We do not sell to patients.
Cas No.: 1910-47-0
Sample solution is provided at 25 µL, 10mM.
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(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.
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