Sodium 3-methyl-2-oxobutanoate
Sodium 3-methyl-2-oxobutanoate structure
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Common Name | Sodium 3-methyl-2-oxobutanoate | ||
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| CAS Number | 3715-29-5 | Molecular Weight | 138.097 | |
| Density | N/A | Boiling Point | 170.2ºC at 760 mmHg | |
| Molecular Formula | C5H7NaO3 | Melting Point | 220-230 °C (dec.)(lit.) | |
| MSDS | Chinese USA | Flash Point | N/A | |
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Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease.
Biochim. Biophys. Acta 1639(3) , 232-8, (2003) Neurological dysfunction is a common finding in patients with maple syrup urine disease (MSUD). However, the mechanisms underlying the neuropathology of brain damage in this disorder are poorly known. In the present study, we investigated the effect of the in... |
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Amino acid metabolism in the human fetus at term: leucine, valine, and methionine kinetics.
Pediatr. Res. 70(6) , 566-71, (2011) Human fetal metabolism is largely unexplored. Understanding how a healthy fetus achieves its fast growth rates could eventually play a pivotal role in improving future nutritional strategies for premature infants. To quantify specific fetal amino acid kinetic... |
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Direct methods and residue type specific isotope labeling in NMR structure determination and model-driven sequential assignment.
J. Biomol. NMR 42(2) , 111-27, (2008) Direct methods in NMR based structure determination start from an unassigned ensemble of unconnected gaseous hydrogen atoms. Under favorable conditions they can produce low resolution structures of proteins. Usually a prohibitively large number of NOEs is req... |
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Engineering Bacillus subtilis for isobutanol production by heterologous Ehrlich pathway construction and the biosynthetic 2-ketoisovalerate precursor pathway overexpression.
Appl. Microbiol. Biotechnol. 91(3) , 577-89, (2011) In the present work, Bacillus subtilis was engineered as the cell factory for isobutanol production due to its high tolerance to isobutanol. Initially, an efficient heterologous Ehrlich pathway controlled by the promoter P(43) was introduced into B. subtilis ... |
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alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats.
Metab. Brain Dis. 20(2) , 155-67, (2005) Maple syrup urine disease (MSUD) is an inherited neurometabolic disorder caused by deficiency of branched-chain alpha-keto acid dehydrogenase complex activity which leads to tissue accumulation of the branched-chain alpha-keto acids (BCKAs) alpha-ketoisocapro... |
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Intrahippocampal administration of the alpha-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats.
Pharmacol. Biochem. Behav. 77(1) , 183-90, (2004) Learning disability is a common feature of patients affected by maple syrup urine disease (MSUD). However, the pathomechanisms underlying learning deficit in this disorder are poorly known. In the present study, we investigated the effect of acute administrat... |
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Branched-chain alpha-keto acids accumulating in maple syrup urine disease induce reorganization of phosphorylated GFAP in C6-glioma cells.
Metab. Brain Dis. 20(3) , 205-17, (2005) In this study we investigate the effects of the branched-chain keto acids (BCKA) alpha-ketoisocaproic (KIC), alpha-ketoisovaleric (KIV), and alpha-keto-beta-methylvaleric (KMV) acids, metabolites accumulating in maple syrup urine disease (MSUD), on the in vit... |
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Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex.
Metab. Brain Dis. 22(1) , 77-88, (2007) The objective of the present study was to investigate the in vitro effects of the branched-chain alpha-keto acids accumulating in maple syrup urine disease, namely L-2-ketoisocaproic acid, L-2-keto-3-methylvaleric acid and L-2-ketoisovaleric acid on Na(+), K(... |
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Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched-chain alpha-keto acids accumulating in maple syrup urine disease.
Cell. Mol. Neurobiol. 26(1) , 67-79, (2006) Accumulation of the branched-chain alpha-keto acids (BCKA), alpha-ketoisocaproic acid (KIC), alpha-keto-beta-methylvaleric acid (KMV), and alpha-ketoisovaleric acid (KIV) and their respective branched-chain alpha-amino acids (BCAA) in tissues and biological f... |
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Alpha-keto-beta-methylvaleric acid increases the in vitro phosphorylation of intermediate filaments in cerebral cortex of young rats through the gabaergic system.
J. Neurol. Sci. 217 , 17-24, (2004) In this study we investigated the effects of alpha-ketoisovaleric (KIV) and alpha-keto-beta-methylvaleric acids (KMV), metabolites accumulating in the inherited neurometabolic disorder maple syrup urine disease (MSUD), on the in vitro incorporation of 32P int... |