Abstract
In order to understand the metabolic characteristics of propionic acidemia, urinary metabolic profiles of six patients (14 days – 5 years old) were studied using gas chromatography-mass spectrometry. All of the patients' urines showed high levels of glycine which correlated inversely to the levels of ketone bodies. The total amounts of propionyl CoA metabolites dramatically increased with the increase in ketone bodies. The amounts of tiglylglycine increased along with the ketone bodies. But a five-year-old boy was almost free from ketosis. It was suggested that production of propionyl CoA as well as acetyl CoA decrease and/or their utilization increases with age. Glycine conjugation of propionyl CoA and tiglyl CoA seems to take place more efficiently as a child grows older.
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