Ethylmalonic acid impairs brain mitochondrial succinate and malate transport

Molecular Genetics and Metabolism

Volumn 105, Issue 1, 2012, Pages 84-90

  Amaral, Alexandre Umpierrez ^a   Cecatto, Cristiane ^a   Busanello, Estela Natasha Brandt ^a   Ribeiro, César Augusto João ^a   Melo, Daniela Rodrigues ^c   Leipnitz, Guilhian ^a   Castilho, Roger Frigério ^c   Wajner, Moacir ^a,b  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

^c UNIVERSITY OF CAMPINAS   (Brazil)

Author keywords

Brain mitochondria; Dicarboxylate transport; Ethylmalonic encephalopathy; Oxidative metabolism; Short chain acyl CoA dehydrogenase deficiency; Succinate

Indexed keywords

ADENOSINE DIPHOSPHATE; ALAMETHICIN; BUMADIZONE; DICARBOXYLATE TRANSPORTER; ETHYLMALONIC ACID; GLUTAMIC ACID; MALIC ACID; MALONIC ACID; METHYLMALONIC ACID; SUCCINATE DEHYDROGENASE; SUCCINIC ACID; UNCLASSIFIED DRUG;

AEROBIC METABOLISM; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BRAIN MITOCHONDRION; BRAIN OXYGEN CONSUMPTION; BUTYRYL COENZYME A DEHYDROGENASE DEFICIENCY; CONTROLLED STUDY; ENZYME DEFICIENCY; ETHYLMALONIC ENCEPHALOPATHY; HYPERLACTATEMIA; IN VITRO STUDY; MITOCHONDRIAL MEMBRANE; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRIAL PERMEABILITY; MITOCHONDRIAL RESPIRATION; NEUROTOXICITY; NONHUMAN; PRIORITY JOURNAL; RAT;

ANIMALS; BIOLOGICAL TRANSPORT; BRAIN; DICARBOXYLIC ACID TRANSPORTERS; MALATES; MALONATES; METHYLMALONIC ACID; MITOCHONDRIA; OXYGEN CONSUMPTION; RATS; RATS, WISTAR; SUCCINIC ACID;

RATTUS;

EID: 84855351431     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2011.10.006     Document Type: Article

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