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11161

Sigma-Aldrich

Sodium (R)-β-hydroxyisobutyrate

≥96.0%

Sinónimos:

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

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About This Item

Fórmula empírica (notación de Hill):
C4H7NaO3
Número de CAS:
1228078-57-6
Peso molecular:
126.09
MDL number:
MFCD17215934
UNSPSC Code:
12352209
PubChem Substance ID:
329749318
NACRES:
NA.25
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Quality Level

100

assay

≥96.0% (GC)
≥96.0%

optical purity

enantiomeric excess: ≥98.0%

composition

sodium, 17.5-19.0%

storage temp.

2-8°C

SMILES string

[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-;/m1./s1

InChI key

RBJZIQZDAZLXEK-AENDTGMFSA-M

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

Biochem/physiol Actions

Increased urinary concentrations of 3-hydroxyisobutyric acid are a biochemical hallmark of metabolic disease 3-hydroxyisobutyric aciduria[1][2].

Packaging

Bottomless glass bottle. Contents are inside inserted fused cone.

Storage Class

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

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Certificados de análisis (COA)

Lot/Batch Number
1448264
1448264V

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(R)-3-Hydroxybutyric acid ≥98.0% (T)

Sigma-Aldrich

54920

(R)-3-Hydroxybutyric acid

(±)-3-hidroxibutirato de sodio ≥99.0% (NT)

Sigma-Aldrich

54965

(±)-3-hidroxibutirato de sodio

Neuropathology of 3-hydroxyisobutyric aciduria, an autopsy case report.
Xianyuan Song et al.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques, 36(4), 483-486 (2009-08-05)
Jörn Oliver Sass et al.
Journal of inherited metabolic disease, 35(3), 437-442 (2011-08-25)
3-hydroxyisobutyric aciduria is an organic aciduria with a poorly understood biochemical basis. It has previously been assumed that deficiency of 3-hydroxyisobutyrate dehydrogenase (HIBADH) in the valine catabolic pathway is the underlying enzyme defect, but more recent evidence makes it likely
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.
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
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