Abstract
Methylmalonic acidaemia and propionic acidaemia are inherited metabolic disorders characterized respectively by methylmalonate (MMA) and propionate (PA) accumulation in tissues of affected patients. Both diseases have severe clinical expression and many undiagnosed patients have a fatal outcome. Those who survive presente neurological dysfunction and a variable degree of neuromotor delay/mental retardation. We investigated the in vitro effects of MMA and PA on CO2 production and lipid biosynthesis in rat brain. Brain prisms of suckling animals were incubated in Krebs Ringer bicarbonate buffer in the presence of [2-14C]-glucose and 5.0 mM MMA or 5.0 mM PA. Controls did not contain the metabolites. After incubation, 14CO2 was trapped into Whatman No. 3 paper filter, lipids were extracted from brain prisms and the radioactivity determined in a scintillation counter. We found that MMA but not PA inhibits signicantly CO2 production from glucose. Lipid biosynthesis was also diminished by MMA but not by PA. These results indicate that MMA may impair brain energy metabolism and normal brain development.
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Wannmacher, C., Dutra, J., Wajner, M. et al. 132 METHYLMALONATE AND PROPIONATE EFFECTS ON CO2 PRODUCTION AND LIPID BIOSYNTHESIS IN BRAIN OF SUCKLING RATS. Pediatr Res 30, 650 (1991). https://doi.org/10.1203/00006450-199112000-00162
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DOI: https://doi.org/10.1203/00006450-199112000-00162