Ketonuria, Branched-chain syrup urine disease

The catabolism of leucine, valine, and isoleucine presents many analogies to fatty acid catabolism. Metabolic disorders of branched-chain amino acid catabolism include hypervalinemia, maple syrup urine disease, intermittent branched-chain ketonuria, isovaleric acidemia, and methylmalonic aciduria. [Pg.262]

Dl. Dancis, J., and Levitz, M., Maple syrup urine disease (branched chain ketonuria). In "The Metabolic Basis of Inherited Disease (J. B. Stanbury, J. B. Wyn-gaarden, and D. S. Fredrickson, eds.), 2nd Ed., pp. 353-365. McGraw-Hill, New York, 1966. [Pg.202]

MAPLE SYRUP URINE DISEASE (BRANCHED-CHAIN KETONURIA) 235... [Pg.235]

Greer, M. and Williams, C.M. (1967), Diagnosis of branched-chain ketonuria (Maple syrup urine disease) by gas chromatography. Biochem. Med., 1,87. [Pg.143]

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