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16842

Sigma-Aldrich

Sodium (S)-β-hydroxyisobutyrate

≥96.0%

Sinonimo/i:

(S)-β-Hydroxy-isobutyric acid sodium salt, (S)-3-Hydroxy-2-methylpropionic acid sodium salt, S-HIBA, R-β-HIBA-Na, Sodium (S)-3-hydroxy-2-methylpropionate

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About This Item

Formula empirica (notazione di Hill):
C4H7NaO3
Numero CAS:
1893416-17-5
Peso molecolare:
126.09
Numero MDL:
MFCD17215930
Codice UNSPSC:
12352106
ID PubChem:
329751395
NACRES:
NA.25

Saggio

≥96.0% (GC)
≥96.0%

Purezza ottica

enantiomeric excess: ≥98.0%

Composizione

sodium, 17.5-19.0%

Temperatura di conservazione

2-8°C

Stringa SMILE

[Na+].C[C@@H](CO)C([O-])=O

InChI

1S/C4H8O3.Na/c1-3(2-5)4(6)7;/h3,5H,2H2,1H3,(H,6,7);/q;+1/p-1/t3-;/m0./s1
RBJZIQZDAZLXEK-DFWYDOINSA-M

Applicazioni

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). (S)-β-hydroxyisobutyrate may be used in stereospecific studies.

Azioni biochim/fisiol

Metabolite in valine, leucine and isoleucine degradation, a reactant of enzymes 3-hydroxyisobutyrate dehydrogenase (EC: 1.1.1.31), 3-hydroxyacyl-CoA dehydrogenase (EC: 1.1.1.35) and 3-hydroxyisobutyryl-CoA hydrolase (EC: 3.1.2.4). Enantiomeric ratio can be used in diagnosis of metabolic diseases, e.g. 3-hydroxyisobutyric aciduria.

Confezionamento

Bottomless glass bottle. Contents are inside inserted fused cone.

Codice della classe di stoccaggio

11 - Combustible Solids

Classe di pericolosità dell'acqua (WGK)

WGK 3

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Certificati d'analisi (COA)

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Sigma-Aldrich

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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.
Ronald J A Wanders et al.
Journal of inherited metabolic disease, 35(1), 5-12 (2010-11-26)
Valine is one of the three branched-chain amino acids which undergoes oxidation within mitochondria. In this paper, we describe the current state of knowledge with respect to the enzymology of the valine oxidation pathway and the different disorders affecting oxidation.
F Podebrad et al.
Clinica chimica acta; international journal of clinical chemistry, 292(1-2), 93-105 (2000-02-25)
The chiral metabolites 3-hydroxyisobutyric acid (HIBA) and 3-aminoisobutyric acid (AIBA) are intermediates in the pathways of L-valine and thymine and play an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (McKusick 236975) and methylmalonic
Tianran Yao et al.
Applied biochemistry and biotechnology, 160(3), 694-703 (2009-06-12)
The MmsB gene product from Bacillus cereus ATCC14579 exhibits 3-hydroxypropionate dehydrogenase activity. It encodes the 32-kDa enzyme protein composed of 292 amino acids. Recombinant 3-hydroxyisobutyrate dehydrogenase (3-HIBADH) was purified 100-fold from cell extract by ammonium sulfate fractionation and column chromatography.

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