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  • Title: A rapid method for assay of branched-chain keto acid decarboxylation in cultured cells and its application to prenatal diagnosis of maple syrup urine disease.
    Author: Fensom AH, Benson PF, Baker JE.
    Journal: Clin Chim Acta; 1978 Jul 01; 87(1):169-74. PubMed ID: 668138.
    Abstract:
    A sensitive assay for measurement of branched-chain keto acid decarboxylation in small numbers of fibroblasts or amniotic cells grown in the wells of a microtitre plate using [1-14C]leucine as substrate is described. The method was applied to the amniotic cells from a pregnancy at risk for maple syrup urine disease and a heterozygous fetus predicted.
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