New conjugated urinary metabolites in intermediate type maple syrup urine disease

  • Clin Chim Acta. 1987 Oct 30;169(1):77-83. doi: 10.1016/0009-8981(87)90395-0.
L Hagenfeldt  1 ,  A S Naglo
Affiliations
  • 1. Department of Clinical Chemistry and Blood Coagulation, Karolinska Hospital, Stockholm, Sweden.
PMID: 3677437 DOI: 10.1016/0009-8981(87)90395-0
Abstract

N-Lactylvaline, N-lactylleucine, N-lactylisoleucine and the N-2-hydroxyisovaleryl conjugates of glycine, valine, leucine and isoleucine have been identified in urine from a patient with the intermediate type of maple syrup urine disease.

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