Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.
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.
D000328:Adult; D000597:Amino Acids, Branched-Chain / Q000097:blood*; D002849:Chromatography, Gas; D006801:Humans; D007651:Keto Acids / Q000097:blood*; D008297:Male; D008375:Maple Syrup Urine Disease / Q000097:blood*
D000597:Amino Acids, Branched-Chain; D007651:Keto Acids