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Sigma-Aldrich

(R)-β-Hydroxy-isobuttersäure Natriumsalz

≥96.0%

Synonym(e):

(R)-3-Hydroxy-2-methyl-propionsäure Natriumsalz, R-HIBA, Natrium-(R)-β-hydroxyisobutyrat, Natrium-(R)-3-hydroxy-2-methylpropionat

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About This Item

Empirische Formel (Hill-System):
C4H7NaO3
CAS-Nummer:
1228078-57-6
Molekulargewicht:
126.09
MDL-Nummer:
MFCD17215934
UNSPSC-Code:
12352209
PubChem Substanz-ID:
329749318
NACRES:
NA.25

Assay

≥96.0% (GC)
≥96.0%

Optische Reinheit

enantiomeric excess: ≥98.0%

Zusammensetzung

sodium, 17.5-19.0%

Lagertemp.

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

InChIKey

RBJZIQZDAZLXEK-AENDTGMFSA-M

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Anwendung

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. Wirkung

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

Verpackung

Bottomless glass bottle. Contents are inside inserted fused cone.

Lagerklassenschlüssel

11 - Combustible Solids

WGK

WGK 3

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable

Analysenzertifikate (COA)

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(R)-3-Hydroxy-buttersäure ≥98.0% (T)

Sigma-Aldrich

54920

(R)-3-Hydroxy-buttersäure

(±)-Natrium 3-Hydroxybuttersäure Natriumsalz ≥99.0% (NT)

Sigma-Aldrich

54965

(±)-Natrium 3-Hydroxybuttersäure Natriumsalz

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)
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.
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
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.
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