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Origin of the two peaks for 2-keto-3-methylvaleric acid produced by the oximation of the keto acids occurring in maple syrup urine disase.

作者信息

Mamer O A, Montgomery J A, Taguchi V Y

出版信息

J Chromatogr. 1980 May 9;182(2):221-5. doi: 10.1016/s0378-4347(00)81626-6.

DOI:10.1016/s0378-4347(00)81626-6
PMID:7380914
Abstract
摘要

相似文献

1
Origin of the two peaks for 2-keto-3-methylvaleric acid produced by the oximation of the keto acids occurring in maple syrup urine disase.枫糖尿症中酮酸肟化产生的2-酮-3-甲基戊酸两个峰的起源。
J Chromatogr. 1980 May 9;182(2):221-5. doi: 10.1016/s0378-4347(00)81626-6.
2
Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.作为肟衍生物分离出的支链α-酮酸：与枫糖尿症中相应的羟基酸和氨基酸的关系。
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3
Determination of alpha-keto acids as silylated oximes in urine and serum by combined gas chromatography-mass spectrometry.
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4
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.枫糖尿症中积累的α-酮酸可显著降低大鼠大脑皮层突触质膜上钠钾ATP酶的活性。
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5
On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans.
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6
Quantitative analysis of branched-chain alpha-keto acids as their trimethylsilylated oximes.
Clin Chim Acta. 1973 Oct 30;48(3):279-85. doi: 10.1016/0009-8981(73)90197-6.
7
Gas-liquid chromatography of alpha-keto acids: quantification of the branched-chain-alpha-keto acids from physiological sources.α-酮酸的气-液色谱法：生理来源中支链α-酮酸的定量分析。
Anal Biochem. 1979 Jan 1;92(1):159-63. doi: 10.1016/0003-2697(79)90638-9.
8
Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease.枫糖尿症中积累的α-酮酸对脑能量代谢的抑制作用。
Biochim Biophys Acta. 2003 Nov 20;1639(3):232-8. doi: 10.1016/j.bbadis.2003.09.010.
9
Intrahippocampal administration of the alpha-keto acids accumulating in maple syrup urine disease provokes learning deficits in rats.
Pharmacol Biochem Behav. 2004 Jan;77(1):183-90. doi: 10.1016/j.pbb.2003.10.013.
10
The gas-chromatographic diagnosis of intermittent maple syrup urine disease (branch-chain ketoaciduria).间歇性枫糖尿症（支链酮酸尿症）的气相色谱诊断
Clin Chim Acta. 1972 Aug;40(1):5-11. doi: 10.1016/0009-8981(72)90244-6.

引用本文的文献

1
Mechanism of biosynthesis of 2-oxo-3-methylvalerate in Chlorobium vibrioforme.绿弯菌中2-氧代-3-甲基戊酸的生物合成机制。
J Bacteriol. 1988 Jul;170(7):3287-90. doi: 10.1128/jb.170.7.3287-3290.1988.