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New lines management of lactic acidosis

J J Gargus, K Boyle, M Bocian, D S Roe, C Vianey-Saban, C R Roe
The mitochondrial oxidative phosphorylation and fatty acid oxidation pathways have traditionally been considered independent major sources of cellular energy production; however, case reports of patients with specific enzymatic defects in either pathway have suggested the potential for a complex interference between the two. This study documents a new site of interference between the two pathways, a site in respiratory complex II capable of producing clinical signs of a block in fatty acid oxidation and reduced in vitro activity of acyl-CoA dehydrogenases...
2003: Journal of Inherited Metabolic Disease
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