2-Hydroxy-3-methylbutyric acid
2-Hydroxy-3-methylbutyric acid structure
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Common Name | 2-Hydroxy-3-methylbutyric acid | ||
|---|---|---|---|---|
| CAS Number | 4026-18-0 | Molecular Weight | 118.131 | |
| Density | 1.1±0.1 g/cm3 | Boiling Point | 237.6±13.0 °C at 760 mmHg | |
| Molecular Formula | C5H10O3 | Melting Point | 86-87ºC(lit.) | |
| MSDS | Chinese USA | Flash Point | 111.8±16.3 °C | |
| Symbol |
GHS07 |
Signal Word | Warning | |
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Physiology and pathophysiology of organic acids in cerebrospinal fluid.
J. Inherit. Metab. Dis. 16(4) , 648-69, (1993) Concentrations of organic acids in cerebrospinal fluid (CSF) appear to be directly dependent upon their rate of production in the brain. There is evidence that the net release of short-chain monocarboxylic acids from the brain is a major route for removing th... |
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α-Ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain
Brain Res. 1324 , 75-84, (2010) Patients affected by maple syrup urine disease (MSUD) present severe neurological symptoms and brain abnormalities, whose pathoiphysiology is poorly known. In the present study we investigated the in vitro effects of leucine (Leu), α-ketoisocaproic acid (KIC)... |
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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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The human cerebrospinal fluid metabolome
J. Chromatogr. B. Analyt. Technol. Biomed. Life Sci. 871(2) , 164-73, (2008) With continuing improvements in analytical technology and an increased interest in comprehensive metabolic profiling of biofluids and tissues, there is a growing need to develop comprehensive reference resources for certain clinically important biofluids, suc... |
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Enantiomeric separation and discrimination of 2-hydroxy acids as O-trifluoroacetylated (S)-(+)-3-methyl-2-butyl esters by achiral dual-capillary column gas chromatography.
J. Chromatogr. A. 874(1) , 91-100, (2000) An efficient method is described for the simultaneous enantiomeric separation of 18 different racemic 2-hydroxy acids for the determination of their absolute configurations. It involves the conversion of each enantiomer into a diastereomeric O-trifluoroacetyl... |
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Proton HR-MAS NMR spectroscopic characterization of metabolites in various human organ tissues: pancreas, brain and liver from trauma cases.
Physiol. Chem. Phys. Med. NMR 40 , 67-88, (2008) High-Resolution Magic Angle Spinning (HR-MAS) NMR has been employed to characterize various metabolites of human pancreas, liver and brain tissues from trauma cases. The potential usefulness of NMR in identifying the metabolites in human tissues has been expl... |
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Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry.
J. Chromatogr. B. Analyt. Technol. Biomed. Life Sci. 775(1) , 115-20, (2002) A new method for quantifying specific amino acids in small volumes of plasma and whole blood has been developed. Volatile derivatives of amino acids are analyzed by gas chromatography-mass spectrometry. The method only takes a few minutes to perform and requi... |
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Bacterial expression of catalytically active fragments of the multifunctional enzyme enniatin synthetase.
J. Mol. Biol. 243(1) , 116-22, (1994) Enniatin synthetase catalyzes the biosynthesis of N-methylated cyclohexadepsipeptides. The 347 kDa enzyme is encoded by the esyn1 gene of Fusarium scirpi and contains two domains (EA and EB) homologous to each other and to regions of other microbial peptide s... |
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Effects of birth asphyxia on urinary organic acid excretion.
Biol. Neonate 61(3) , 162-72, (1992) Using capillary gas chromatography-mass spectrometry, the effects of birth asphyxia on the urinary organic acid profile of term babies was investigated. Random urine samples were collected on days 1 and 8 from 19 babies with fetal distress, 19 with moderate b... |
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Disturbances of valine metabolism in patients with peroxisomal biogenesis disorders.
Adv. Exp. Med. Biol. 544 , 73-4, (2003)
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