Maple syrup urine disease: coenzyme function and prenatal monitoring.

Adult Amino Acids/metabolism Amniotic Fluid/analysis,cytology Carbon Dioxide/metabolism Carbon Radioisotopes Cells, Cultured Coenzyme A/physiology Decarboxylation Fasting Female Fibroblasts Genotype Heterozygote Humans Infant, Newborn Isoleucine/analysis,blood,urine Leucine/analysis,blood,urine Maple Syrup Urine Disease/diagnosis,enzymology Maternal-Fetal Exchange Pregnancy Prenatal Diagnosis Radioimmunoassay Valine/analysis,blood,urine

Amino Acids Carbon Radioisotopes Isoleucine Carbon Dioxide Leucine Valine Coenzyme A