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Clinical Chemistry 18: 413-416, 1972;
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Clinical Chemistry, Vol 18, 413-416, Copyright © 1972 by the American Association for Clinical Chemistry

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

Richard J. Allen 1, H. Jeane Frey 1, LaDona M. Fleming 1, and Clyde L. Owings 1

1 Section of Pediatric Neurology, Department of Pediatrics, University of Michigan Medical Center, Ann Arbor, Mich. 48104.

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 Words: diagnostic aid • monitoring of treatment • branched-chain amino acids in biological fluids

Submitted on January 3, 1972
Accepted on February 16, 1972






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Copyright © 1972 by the American Association for Clinical Chemistry.