Interrelation between the Metabolism of L-Isoleucine and L-Allo-Isoleucine in Patients with Maple Syrup Urine Disease
作者:
UDO WENDEL,
ULRICH LANGENBECK,
JAMES SEAKINS,
期刊:
Pediatric Research
(OVID Available online 1989)
卷期:
Volume 25,
issue 1
页码: 11-14
ISSN:0031-3998
年代: 1989
出版商: OVID
数据来源: OVID
摘要:
The nonprotein amino acid L-allo-isoleucine is formed endogenously in maple syrup urine disease patients from (R)-3-methyl-2-oxo-pentanoic acid. During strict metabolic balance, the plasma L-allo-isoleucine/L-isoleucine ratio correlates inversely with the residual activity of the branched-chain 2-oxoacid dehydrogenase in fibroblasts and thus constitutes a relevantin vivoparameter of the severity of the metabolic defect in MSUD patients.
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