(R)-3-Hydroxyisobutyric acid |
| Katalog-Nr.GC30661 |
(R)-3-HydroxyisobuttersÄure ist ein Zwischenprodukt in den Signalwegen von L-Valin und Thymin und spielt eine wichtige Rolle bei der Diagnose der sehr seltenen erblichen Stoffwechselerkrankungen 3-HydroxyisobuttersÄure-Azidurie und Methylmalon-Semialdehyd-Dehydrogenase-Mangel.
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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