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

2-Keto-3-methylvaleric acid (KMVA) has been found not to undergo spontaneous keto-enol tautomerization in neutral aqueous solution, alone or in the presence of large concentrations of pyridoxamine or pyridoxamine-5-phosphate. This finding denies the commonly held suppositions that 3R-KMVA is derived...

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Veröffentlicht in:	The Journal of biological chemistry 1992-11, Vol.267 (31), p.22141-22147
Hauptverfasser:	MAMER, O. A, REIMER, M. L. J
Format:	Artikel
Sprache:	eng
Schlagworte:	Aminoacids, peptides. Hormones. Neuropeptides Analytical, structural and metabolic biochemistry Biological and medical sciences Chromatography, Gas Fundamental and applied biological sciences. Psychology Gas Chromatography-Mass Spectrometry Humans Isoleucine - chemistry Isoleucine - metabolism Magnetic Resonance Spectroscopy Maple Syrup Urine Disease - metabolism Pentanoic Acids - chemistry Pentanoic Acids - metabolism Proteins Stereoisomerism
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