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| Brusque,A.M.; Rotta,L.; Pettenuzzo,L.F.; Junqueira,D.; Schwarzbold,C.V.; Wyse,A.T.; Wannmacher,C.M.D.; Dutra-Filho,C.S.; Wajner,M.. |
| Levels of methylmalonic acid (MMA) comparable to those of human methylmalonic acidemia were achieved in blood (2-2.5 mmol/l) and brain (1.35 µmol/g) of rats by administering buffered MMA, pH 7.4, subcutaneously twice a day from the 5th to the 28th day of life. MMA doses ranged from 0.76 to 1.67 µmol/g as a function of animal age. Control rats were treated with saline in the same volumes. The animals were sacrificed by decapitation on the 28th day of age. Blood was taken and the brain was rapidly removed. Medulla, pons, the olfactory lobes and cerebellum were discarded and the rest of the brain ("cerebrum") was isolated. Body and "cerebrum" weight were measured, as well as the cholesterol and triglyceride concentrations in blood and the content of myelin,... |
| Tipo: Info:eu-repo/semantics/other |
Palavras-chave: Methylmalonic acid; Glycerides; Lipids; Myelin; Rat brain; Methylmalonic acidemia. |
| Ano: 2001 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2001000200010 |
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| Silva,A.R.; Ruschel,C.; Helegda,C.; Wyse,A.T.S.; Wannmacher,C.M.D.; Wajner,M.; Dutra-Filho,C.S.. |
| 2-Hydroxybutyric acid appears at high concentrations in situations related to deficient energy metabolism (e.g., birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. The present study was carried out to determine the effect of 2-hydroxybutyric acid at various concentrations (1-10 mM) on CO2 production and lipid synthesis from labeled substrates in cerebral cortex of 30-day-old Wistar rats in vitro. CO2 production was significantly inhibited (30-70%) by 2-hydroxybutyric acid in cerebral cortex prisms, in total homogenates and in the mitochondrial fraction. We... |
| Tipo: Info:eu-repo/semantics/other |
Palavras-chave: Lipid synthesis; Energy metabolism; Cerebral cortex; 2-hydroxybutyric acid. |
| Ano: 2001 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2001000500010 |
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