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Background:  The determination of branched chain amino acids [BCAA; valine (Val), leucine (Leu), isoleucine (Ile)], α-keto acids derived from BCAA [BCKA; α-ketoisovaleric acid (KIV), α-ketoisocaproic acid (KIC), α-ketomethylvaleric acid (KMV)], methionine (Met), phenylalanine (Phe) and tyrosine (Tyr) is currently the most reliable approach for the diagnosis of maple syrup urine disease (MSUD), hypermethioninemia, phenylketonuria (PKU) and tyrosinemia. The aim of this study was to develop rapid and simple HPLC methods for measurement of BCAA, Met, Phe, Tyr and BCKA in plasma and dried blood samples.

clinical chemistry and laboratory medicine

Determination of branched chain amino acids, methionine, phenylalanine, tyrosine and α-keto acids in plasma and dried blood samples using HPLC with fluorescence detection