Metabolite Profiles Reveal Energy Failure and Impaired Beta-Oxidation in Liver of Mice with Complex III Deficiency Due to a BCS1L Mutation

BACKGROUND & AIMS: Liver is a target organ in many mitochondrial disorders, especially if the complex III assembly factor BCS1L is mutated. To reveal disease mechanism due to such mutations, we have produced a transgenic mouse model with c.232A>G mutation in Bcs1l, the causative mutation for...

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Main Authors: Kotarsky, Heike, Keller, Matthias, Davoudi, Mina, Levéen, Per, Karikoski, Riitta, Enot, David P., Fellman, Vineta
Formato: Artigo
Idioma:Inglês
Publicado: Public Library of Science 2012
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Research Article
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC3400604/
https://ncbi.nlm.nih.gov/pubmed/22829922
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0041156
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