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Plasma amino acid analyses in two cases of maple syrup urine disease.
Southeast Asian J Trop Med Public Health. 1999; 30 Suppl 2:138-9.SA

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.

Authors+Show Affiliations

Surarit R
Laboratory of Biochemistry, Chulabhorn Research Institute, Bangkok, Thailand.
Srisomsap C
No affiliation info available
Wasant P
No affiliation info available
Svasti J
No affiliation info available
Suthatvoravut U
No affiliation info available
Chokchaichamnankit D
No affiliation info available
Liammongkolkul S
No affiliation info available

MeSH

Amino AcidsHumansInfantInfant, NewbornIsoleucineLeucineMaleMaple Syrup Urine DiseaseValine

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

11400750

Citation

Surarit, R, et al. "Plasma Amino Acid Analyses in Two Cases of Maple Syrup Urine Disease." The Southeast Asian Journal of Tropical Medicine and Public Health, vol. 30 Suppl 2, 1999, pp. 138-9.
Surarit R, Srisomsap C, Wasant P, et al. Plasma amino acid analyses in two cases of maple syrup urine disease. Southeast Asian J Trop Med Public Health. 1999;30 Suppl 2:138-9.
Surarit, R., Srisomsap, C., Wasant, P., Svasti, J., Suthatvoravut, U., Chokchaichamnankit, D., & Liammongkolkul, S. (1999). Plasma amino acid analyses in two cases of maple syrup urine disease. The Southeast Asian Journal of Tropical Medicine and Public Health, 30 Suppl 2, 138-9.
Surarit R, et al. Plasma Amino Acid Analyses in Two Cases of Maple Syrup Urine Disease. Southeast Asian J Trop Med Public Health. 1999;30 Suppl 2:138-9. PubMed PMID: 11400750.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Plasma amino acid analyses in two cases of maple syrup urine disease. AU - Surarit,R, AU - Srisomsap,C, AU - Wasant,P, AU - Svasti,J, AU - Suthatvoravut,U, AU - Chokchaichamnankit,D, AU - Liammongkolkul,S, PY - 2001/6/13/pubmed PY - 2001/7/13/medline PY - 2001/6/13/entrez SP - 138 EP - 9 JF - The Southeast Asian journal of tropical medicine and public health JO - Southeast Asian J Trop Med Public Health VL - 30 Suppl 2 N2 - 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. SN - 0125-1562 UR - https://www.unboundmedicine.com/medline/citation/11400750/Plasma_amino_acid_analyses_in_two_cases_of_maple_syrup_urine_disease_ L2 - https://www.diseaseinfosearch.org/result/4453 DB - PRIME DP - Unbound Medicine ER -