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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:
12352209
MDL number:
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
SMILES string
[Na+].C[C@H](CO)C([O-])=O
InChI key
RBJZIQZDAZLXEK-AENDTGMFSA-M
assay
≥96.0% (GC), ≥96.0%
optical purity
enantiomeric excess: ≥98.0%
composition
sodium, 17.5-19.0%
storage temp.
2-8°C
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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.
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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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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