New conjugated urinary metabolites in intermediate type maple syrup urine disease.
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.
Hagenfeldt
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L
Department of Clinical Chemistry and Blood Coagulation, Karolinska Hospital, Stockholm, Sweden.
Naglo
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AS
D000596:Amino Acids / Q000652:urine*; D000597:Amino Acids, Branched-Chain / Q000652:urine; D002264:Carboxylic Acids / Q000652:urine; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D005998:Glycine / Q000652:urine; D006801:Humans; D007223:Infant; D007773:Lactates / Q000652:urine; D019344:Lactic Acid; D008375:Maple Syrup Urine Disease / Q000652:urine*; D014631:Valerates / Q000652:urine
D000596:Amino Acids; D000597:Amino Acids, Branched-Chain; D002264:Carboxylic Acids; D007773:Lactates; D014631:Valerates; C035864:2-hydroxyisovaleric acid; D019344:Lactic Acid; D005998:Glycine