Sodium(R)-β-hydroxyisobutyrate
Sodium(R)-β-hydroxyisobutyrate structure
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Common Name | Sodium(R)-β-hydroxyisobutyrate | ||
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| CAS Number | 1228078-57-6 | Molecular Weight | 126.08600 | |
| Density | N/A | Boiling Point | N/A | |
| Molecular Formula | C4H7NaO3 | Melting Point | N/A | |
| MSDS | USA | Flash Point | N/A | |
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Stereodifferentiation of 3-hydroxyisobutyric- and 3-aminoisobutyric acid in human urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry.
Clin. Chim. Acta 292 , 93-105, (2000) 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 ac... |
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Enzymology of the branched-chain amino acid oxidation disorders: the valine pathway.
J. Inherit. Metab. Dis. 35 , 5-12, (2012) 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... |
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Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.
Am. J. Hum. Genet. 80 , 195-199, (2007) 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-hydroxyiso... |
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Expression of 3-hydroxyisobutyrate dehydrogenase in cultured neural cells.
J. Neurochem. 105 , 1176-1186, (2008) 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 parenc... |
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The catalytic property of 3-hydroxyisobutyrate dehydrogenase from Bacillus cereus on 3-hydroxypropionate.
Appl. Biochem. Biotechnol. 160 , 694-703, (2010) 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 ce... |
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3-Hydroxyisobutyrate aciduria and mutations in the ALDH6A1 gene coding for methylmalonate semialdehyde dehydrogenase.
J. Inherit. Metab. Dis. 35 , 437-442, (2012) 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 ... |
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Neuropathology of 3-hydroxyisobutyric aciduria, an autopsy case report.
Can J Neurol Sci 36 , 483-486, (2009)
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