Mitochondrial 2,4-dienoyl-CoA Reductase Deficiency in Mice Results in Severe Hypoglycemia with Stress Intolerance and Unimpaired Ketogenesis

The mitochondrial β-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 β-oxidation of uns...

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Detalles Bibliográficos
Autores principales: Miinalainen, Ilkka J., Schmitz, Werner, Huotari, Anne, Autio, Kaija J., Soininen, Raija, Ver Loren van Themaat, Emiel, Baes, Myriam, Herzig, Karl-Heinz, Conzelmann, Ernst, Hiltunen, J. Kalervo
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2009
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2697383/
https://www.ncbi.nlm.nih.gov/pubmed/19578400
http://dx.doi.org/10.1371/journal.pgen.1000543

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