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Title: Plasma amino acid analyses in two cases of maple syrup urine disease
Authors: Rudee Surarit
Chantragan Srisomsap
Pornswan Wasant
Jisnuson Svasti
Umaporn Suthatvoravut
Daranee Chokchaichamnankit
Somporn Liammongkolkul
Chulabhorn Research Institute
Mahidol University
Keywords: Medicine
Issue Date: 1-Dec-1999
Citation: Southeast Asian Journal of Tropical Medicine and Public Health. Vol.30, No.SUPPL. 2 (1999), 138-139
Abstract: Maple syrup urine disease is a rare inborn error of metabolism, characterized by elevated plasma levels of branched chain amino acids and urinary excretion of branched chain keto acids. Plasma amino acid levels in two subjects were followed by deproteinizing plasma, derivatizing the free amino acids with phenylisothiocyanate, and analysis by HPLC. The results indicate that valine, leucine and isoleucine are elevated in Maple syrup urine disease, and that leucine remains high even after dietary treatment.
ISSN: 01251562
Appears in Collections:Scopus 1991-2000

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