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Semiquantitation of Leucine, Isoleucine, and Valine by Thin-Layer Chromatography in Management of Maple-Syrup Urine Disease

Richard J. Allen, H.J. Frey, Ladona M. Fleming, Clyde L. Owings

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Abstract

Abstract A few microliters of serum suffice to estimate the leucine, isoleucine, and valine present. The three branched-chain amino acids are separated by unidirectional thin-layer chromatography, and estimated by visual comparison with co-chromatographed individual standards in graduated concentrations. The method is especially applicable to the frequent monitoring of serum branchedchain amino acids needed in treating patients with maple-syrup urine disease, and may also be used for rapid diagnosis of the disease in suspected infants.

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What this paper is about

Abstract A few microliters of serum suffice to estimate the leucine, isoleucine, and valine present. The three branched-chain amino acids are separated by unidirectional thin-layer chromatography, and estimated by visual comparison with co-chromatographed individual standards in graduated concentrations. The method is especially applicable to the frequent monitoring of serum branchedchain amino acids needed in treating patients with maple-syrup urine disease, and may also be used for rapid diagnosis of the disease in suspected infants.

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Available abstract

Abstract A few microliters of serum suffice to estimate the leucine, isoleucine, and valine present. The three branched-chain amino acids are separated by unidirectional thin-layer chromatography, and estimated by visual comparison with co-chromatographed individual standards in graduated concentrations. The method is especially applicable to the frequent monitoring of serum branchedchain amino acids needed in treating patients with maple-syrup urine disease, and may also be used for rapid diagnosis of the disease in suspected infants.

Key concepts: Maple syrup urine disease, Valine, Leucine, Isoleucine, Urine, Maple, Amino acid, Chromatography

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Semiquantitation of Leucine, Isoleucine, and Valine by Thin-Layer Chromatography in Management of Maple-Syrup Urine Disease — Research Paper | ScholarLens