sodium 3-methyl-2-oxovalerate
sodium 3-methyl-2-oxovalerate structure
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Common Name | sodium 3-methyl-2-oxovalerate | ||
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| CAS Number | 3715-31-9 | Molecular Weight | 152.12400 | |
| Density | N/A | Boiling Point | N/A | |
| Molecular Formula | C6H9NaO3 | Melting Point | 204-206 ℃(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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Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.
J. Inherit. Metab. Dis. 22(6) , 706-22, (1999) In maple syrup urine disease (MSUD), branched-chain L-amino (BCAA) and 2-oxo acids (BCOA) accumulate in body fluids owing to an inherited deficiency of branched-chain 2-oxo acid dehydrogenase complex activity. In MSUD, little information is available on the s... |
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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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Analysis of (S)- and (R)-3-methyl-2-oxopentanoate enantiomorphs in body fluids.
Meth. Enzymol. 324 , 33-9, (2000)
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Chloride-dependent inhibition of vesicular glutamate uptake by alpha-keto acids accumulated in maple syrup urine disease.
Biochim. Biophys. Acta 1475(2) , 114-8, (2000) Maple syrup urine disease is a metabolic disorder caused by mutations of the branched chain keto acid dehydrogenase complex, leading to accumulation of alpha-keto acids and their amino acid precursors in the brain. We now report that alpha-ketoisovaleric, alp... |
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Mycobacterium tuberculosis ketopantoate hydroxymethyltransferase: tetrahydrofolate-independent hydroxymethyltransferase and enolization reactions with alpha-keto acids.
Biochemistry 42(1) , 191-9, (2003) The panB gene that encodes ketopantoate hydroxymethyltransferase has been cloned from Mycobacterium tuberculosis, expressed, and purified to homogeneity. 1H NMR spectroscopy was used to determine the rate of (i) tetrahydrofolate-independent hydroxymethyltrans... |
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Metabolic and K(ATP) channel-independent actions of keto acid initiators of insulin secretion.
Pancreas 20(1) , 38-46, (2000) Insulin-releasing effects of 2-ketobutyric acid (KB), 2-ketoisocaproic acid (KIC), 2-keto-3-methylvaleric acid (KMV), and 3-phenylpyruvic acid (PP) were examined by using clonal beta cells. Whereas KIC, KMV, and PP dose-dependently initiated insulin secretion... |
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Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats.
Brain Res. 894(1) , 68-73, (2001) Neurological dysfunction is common in patients with maple syrup urine disease (MSUD). However, the mechanisms underlying the pathophysiology of this disorder are poorly known. In the present study we investigated the effect of intrastriatal administration of ... |
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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... |