Effect of methylmalonate on ketone body metabolism in developing rat brain |
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Authors: | Mulchand S. Patel Oliver E. Owen Cindy Raefsky |
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Affiliation: | Departments of Medicine and Biochemistry, General Clinical Research Center and Fels Research Institute, Temple University School of Medicine, Philadelphia, Pa. 19140, USA |
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Abstract: | The oxidation of 3-hydroxy[3-14C]butyrate to CO2 and its incorporation into cerebral lipids by cortex slices from one-week old rats were markedly inhibited by methylmalonate. However, methylmalonate had no effect on the metabolism of labelled aceto- acetate, glucose and acetate by brain slices. Addition of propionate in the incubation medium reduced cerebral lipogenesis from labelled 3-hydroxybutyrate and acetate. Acute methylmalonic acidemia induced in one-week old pups by injecting 3% methylmalonate solution caused a reduction in the incorporation of labelled 3-hydroxybutyrate into cerebral lipids. However, acute methylmalonic acidemia had no effect on cerebral lipogensis from labelled acetate. These findings show (i) the conversion of 3-hydroxybutyrate to acetoacetate by 3-hydroxybutyrate dehydrogenase in the brain is inhibited by methylmalonate, and (ii) an inhibition of cerebral lipid synthesis by propionate, which also accumulates in patients with methylmalonic aciduria. |
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