89517
(S)-3,4-Dihydroxybutyric acid lithium salt hydrate
≥95.0% (GC)
Synonym(s):
(S)-3,4-Dihydroxybutanoic acid lithium salt hydrate
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Quality Level
Assay
≥85% (free acid, qNMR)
≥95.0% (GC)
form
solid
optical activity
[α]/D -25±5°, 24 hr, c = 0.1 in 0.1 M HCl
storage temp.
−20°C
SMILES string
O[C@H](CO)CC(O)=O
InChI
1S/C4H8O4/c5-2-3(6)1-4(7)8/h3,5-6H,1-2H2,(H,7,8)/t3-/m0/s1
InChI key
DZAIOXUZHHTJKN-VKHMYHEASA-N
Biochem/physiol Actions
3,4-Dihydroxybutyric acid is a normal human urinary metabolite that is excreted in increased concentration in patients with succinic semialdehyde dehydro- genase (SSADH) deficiency, while healthy adults excrete 0.37 +/- 0.15 (SD) mmoles of 3,4- dihydroxybutyrate per 24 hr. The renal clearance of this compound is roughly equal that of creatinine. This metabolite is also detectable in blood.
Storage Class Code
11 - Combustible Solids
WGK
WGK 3
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We describe the rapid and sensitive detection of 4-hydroxybutyric acid, which is a marker compound for succinic semialdehyde dehydrogenase (SSADH) deficiency. Urinary 4-hydroxybutyric acid and 3,4-dihydroxybutyric acid were targeted, quantified by gas chromatography-mass spectrometry after simplified urease digestion in which
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The occurrence of (S)-3,4-dihydroxybutyrate in human blood and urine.
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Lesch-Nyhan syndrome (LNS) is caused by a severe deficiency of hypoxanthine-guanine phosphoribosyltransferase (HPRT) and clinically characterized by self-injurious behavior and nephrolithiasis; the latter is treatable with allopurinol, an inhibitor of xanthine oxidase which converts xanthine and hypoxanthine into uric acid.
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