36105
(±)-Sodium β-hydroxyisobutyrate
≥96.0%
Synonym(s):
β-Hydroxyisobutyric acid sodium salt, (±)-β-HIBA-Na, 3-Hydroxy-2-methylpropionic acid sodium salt, Sodium 3-hydroxy-2-methylpropionate
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About This Item
Empirical Formula (Hill Notation):
C4H7NaO3
CAS Number:
Molecular Weight:
126.09
NACRES:
NA.25
PubChem Substance ID:
UNSPSC Code:
12352106
MDL number:
InChI
1S/C4H8O3.Na/c1-3(2-5)4(6)7;/h3,5H,2H2,1H3,(H,6,7);/q;+1/p-1
SMILES string
[Na+].CC(CO)C([O-])=O
InChI key
RBJZIQZDAZLXEK-UHFFFAOYSA-M
assay
≥96.0% (GC), ≥96.0%
form
powder
composition
sodium, 17.5-19.0%
storage temp.
2-8°C
Quality Level
Application
3-Hydroxyisobutyrate (HIBA) may be used to study the distribution, characterization and kinetics of enzymes involved in branched chain amino acid metabolism such as 3-hydroxyisobutyrate dehydrogenase (EC: 1.1.1.31) and 3-hydroxyisobutyryl-CoA hydrolase (EC: 3.1.2.4).
Biochem/physiol Actions
3-Hydroxyisobutyrate (HIBA), is formed in the valine catabolism, it is a reactant of enzyme 3-hydroxyisobutyrate dehydrogenase (EC: 1.1.1.31) and 3-hydroxyisobutyryl-CoA hydrolase (EC: 3.1.2.4); it was found to have increased concentrations in MS patient metabolic profiles.
Packaging
Bottomless glass bottle. Contents are inside inserted fused cone.
Storage Class
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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Mammalian 3-hydroxyisobutyrate dehydrogenase.
J W Hawes et al.
Methods in enzymology, 324, 218-228 (2000-09-16)
Disorders of the metabolism of amino acids and related compounds.
Shih, V. E. and Mandell, R. et al.
Genetic Disorders and the Fetus, 514-553 (2010)
Norbert W Lutz et al.
Biochemical and biophysical research communications, 354(1), 160-164 (2007-01-16)
(1)H NMR spectroscopy of cerebrospinal fluid (CSF) is currently being used to study metabolic profiles characteristic of distinct multiple sclerosis (MS) manifestations. For select MS patient groups, we have previously detected significantly increased concentrations of several identified metabolites and one
Ference J Loupatty et al.
American journal of human genetics, 80(1), 195-199 (2006-12-13)
Only a single patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency has been described in the literature, and the molecular basis of this inborn error of valine catabolism has remained unknown until now. Here, we present a second patient with 3-hydroxyisobutyryl-CoA hydrolase deficiency
Radovan Murín et al.
Journal of neurochemistry, 105(4), 1176-1186 (2008-02-21)
The branched-chain amino acids (BCAAs)--isoleucine, leucine, and valine--belong to the limited group of substances transported through the blood-brain barrier. One of the functions they are thought to have in brain is to serve as substrates for meeting parenchymal energy demands.
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