Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/199268
Type: Artigo de periódico
Title: Mitochondrial Energy Metabolism In Neurodegeneration Associated With Methylmalonic Acidemia.
Author: Melo, Daniela R
Kowaltowski, Alicia J
Wajner, Moacir
Castilho, Roger F
Abstract: Methylmalonic acidemia is one of the most prevalent inherited metabolic disorders involving neurological deficits. In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarizes changes in mitochondrial energy metabolism occurring in methylmalonic acidemia, focusing mainly on the effects of accumulated methylmalonic acid, and gives an overview of the results found in different experimental models. Overall, experiments to date suggest that mitochondrial impairment in this disease occurs through a combination of the inhibition of specific enzymes and transporters, limitation in the availability of substrates for mitochondrial metabolic pathways and oxidative damage.
Subject: Amino Acid Metabolism, Inborn Errors
Energy Metabolism
Humans
Methylmalonic Acid
Mitochondria
Neurodegenerative Diseases
Oxygen Consumption
Rights: fechado
Identifier DOI: 10.1007/s10863-011-9330-2
Address: http://www.ncbi.nlm.nih.gov/pubmed/21271280
Date Issue: 2011
Appears in Collections:Artigos e Materiais de Revistas Científicas - Unicamp

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