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To develop a safe and noninvasive in vivo assay of hepatic propionate oxidative capacity. A modified 1- C-propionate breath test was administered to 57 methylmalonic acidemia (MMA) subjects, including 19 transplant recipients, and 16 healthy volunteers. Isotopomer enrichment ( CO / CO ) was measured in exhaled breath after an enteral bolus of sodium-1- C-propionate, and normalized for CO production. 1- C-propionate oxidation was then correlated with clinical, laboratory, and imaging parameters collected via a dedicated natural history
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