Publication: Plasma amino acid analyses in two cases of maple syrup urine disease
6
Issued Date
1999-12-01
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ISSN
01251562
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2-s2.0-0033302055
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Mahidol University
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SCOPUS
Bibliographic Citation
Southeast Asian Journal of Tropical Medicine and Public Health. Vol.30, No.SUPPL. 2 (1999), 138-139
Suggested Citation
Rudee Surarit, Chantragan Srisomsap, Pornswan Wasant, Jisnuson Svasti, Umaporn Suthatvoravut, Daranee Chokchaichamnankit, Somporn Liammongkolkul Plasma amino acid analyses in two cases of maple syrup urine disease. Southeast Asian Journal of Tropical Medicine and Public Health. Vol.30, No.SUPPL. 2 (1999), 138-139. Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/25562
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Title
Plasma amino acid analyses in two cases of maple syrup urine disease
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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.