Abnormal Glucose Metabolism in Skin Fibroblasts Cultured from a Patient with a New Syndrome of Ketoacidemia

Extract: Studies of substrate oxidation by cultured skin fibroblasts from an infant (CC) with severe recurrent episodes of ketoacidosis showed normal rates of oxidation of pro-pionate (CC 55. ± 8 versus control 65 ± 13 μμmoles/mg protein/hr), and succinate (73. ± 16 versus 45 ± 8 μμmoles/mg protein/hr).