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Significance of L-alloisoleucine in plasma for diagnosis of maple syrup urine disease.
BACKGROUND The significance of plasma L-alloisoleucine, which is derived from L-isoleucine in vivo, for diagnosis of maple syrup urine disease (MSUD) was examined. METHODS Branched-chain L-aminoExpand
Formation of L-Alloisoleucine In Vivo : An L-[13C]Isoleucine Study in Man
L-Alloisoleucine (2 S, 3 R), a diastereomer of L-isoleucine (2 S, 3 S), is a normal constituent of human plasma. Considerable amounts accumulate in maple syrup urine disease, in which theExpand
Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation
We characterized the effect of orthotopic liver transplantation on the catabolism of branched-chain L-amino acids in a female patient with classical form of maple syrup urine disease. TransplantationExpand
Whole-Body l-Leucine Oxidation in Patients with Variant Form of Maple Syrup Urine Disease
Whole-body l-leucine oxidation was assessed in patients with maple syrup urine disease of different severity using oral l-[1-13C]leucine bolus tests (38 μmol/kg body weight). Residual whole-bodyExpand
Analysis of concentration and (13)C enrichment of D-galactose in human plasma.
BACKGROUND A stable-isotope dilution method for the sensitive determination of D-galactose in human plasma was established. METHODS D-[(13)C]Galactose was added to plasma, and the concentration wasExpand