Mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis.

Mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis.

The mitochondrial beta-oxidation system is one of the central metabolic pathways of energy metabolism in mammals. Enzyme defects in this pathway cause fatty acid oxidation disorders. To elucidate the role of 2,4-dienoyl-CoA reductase (DECR) as an auxiliary enzyme in the mitochondrial beta-oxidation...

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Main Authors: Ilkka J Miinalainen, Werner Schmitz, Anne Huotari, Kaija J Autio, Raija Soininen, Emiel Ver Loren van Themaat, Myriam Baes, Karl-Heinz Herzig, Ernst Conzelmann, J Kalervo Hiltunen
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-07-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC2697383?pdf=render

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http://europepmc.org/articles/PMC2697383?pdf=render

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