(R)-3-Hydroxyisobutyric acid |
| رقم الكتالوجGC30661 |
(R) -3-Hydroxyisobutyric acid هو وسيط في مسارات L-valine و thymine ويلعب دورًا مهمًا في تشخيص الأمراض الأيضية النادرة جدًا الموروثة 3-hydroxyisobutyric aciduria و methylmalonic semialdehyde dehydrogenase deficiency
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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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