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. 1978 Sep 1;88(2):283-91.
doi: 10.1016/0009-8981(78)90433-3.

Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease

U Langenbeck, U Wendel, A Mench-Hoinowski, D Kuschel, K Becker, H Przyrembel, H J Bremer

Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease

U Langenbeck et al. Clin Chim Acta. .

Abstract

In 62 blood samples from 3 patients with classical maple syrup urine disease and from one patient with a variant form, a close linear correlation was found between levels of branched chain amino acids and their corresponding alpha-keto acids. Keto acids were determined as O-trimethylsilyl quinoxalinols by gas chromatography with a nitrogen-selective detector.

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