caa a22 4500 475792971 CHVBK 20180406123715.0 cr unu---uuuuu 170329e20000601xx s 000 0 eng 10.1007/BF02679979 doi (NATIONALLICENCE)springer-10.1007/BF02679979 Inhibition of energy production in vitro by glutaric acid in cerebral cortex of young rats [Elektronische Daten] [C. Silva, A. Silva, C. Ruschel, C. Helegda, A. Wyse, C. Wannmacher, C. Dutra-Filho, M. Wajner] The present study investigated the effects of glutaric acid (GA), which predominantly accumulates in glutaric acidemia type I (GA-I), on somein vitro parameters of energy metabolism in cerebral cortex of rats. We first evaluated CO2 production from [U-14C] acetate, as well as ATP levels in brain of young Wistar rats. The effect of the acid on the activities of the respiratory chain complexes were also investigated. GA was tested at final concentrations ranging from 0.5 to 5.0 mM. GA significantly reduced brain CO2 production by 50% at the concentrations of 0.5 to 3.0 mM, ATP levels by 25% at the concentration of 3.0 mM, succinate:cytochrome C oxireductase (complex II plus CoQ plus complex III) by 25% at 5 mM concentration, and NADH:cytochrome C oxireductase (complex I plus CoQ plus complex III) by 25% at 2.5 and 5 mM concentrations. The results strongly indicate that GA impairs brain energy production. If these effects also occur in humans, it is possible that they may contribute to the neuropathology of patients affected by GA-I. Plenum Publishing Corporation, 2000 Organic acids nationallicence energy metabolism nationallicence cerebral cortex nationallicence glutaric acid nationallicence glutaric acidemia type I nationallicence Silva C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Silva A. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Ruschel C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Helegda C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Wyse A. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Wannmacher C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Dutra-Filho C. aut Wajner M. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut Metabolic Brain Disease Springer US 15/2(2000-06-01), 123-131 0885-7490 15:2<123 2000 15 11011 https://doi.org/10.1007/BF02679979 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF02679979 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Silva C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 700 1- Silva A. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 700 1- Ruschel C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 700 1- Helegda C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 700 1- Wyse A. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 700 1- Wannmacher C. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 700 1- Dutra-Filho C. aut NATIONALLICENCE 700 1- Wajner M. Departamento de Bioquímica-ICBS, Universidade Federal do Rio Grande do Sul Rua Ramiro Barcelos, 2600 Anexo 900035-003, Porto Alegre, RS, Brazil aut NATIONALLICENCE 773 0- Metabolic Brain Disease Springer US 15/2(2000-06-01), 123-131 0885-7490 15:2<123 2000 15 11011 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer