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11161

(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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Über diesen Artikel

Empirische Formel (Hill-System):
C4H7NaO3
CAS-Nummer:
Molekulargewicht:
126.09
NACRES:
NA.25
PubChem Substance ID:
UNSPSC Code:
12352209
MDL number:
Assay:
≥96.0% (GC), ≥96.0%


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

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.


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Lagerklasse

11 - Combustible Solids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable



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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)
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



Global Trade Item Number

SKUGTIN
11161-100MG04061833238721

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