Impaired mitophagy links mitochondrial disease to epithelial stress in methylmalonyl-CoA mutase deficiency

Deregulation of mitochondrial network in terminally differentiated cells contributes to a broad spectrum of disorders. Methylmalonic acidemia (MMA) is one of the most common inherited metabolic disorders, due to deficiency of the mitochondrial methylmalonyl-coenzyme A mutase (MMUT). How MMUT deficie...

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Publicat a:Nat Commun
Autors principals: Luciani, Alessandro, Schumann, Anke, Berquez, Marine, Chen, Zhiyong, Nieri, Daniela, Failli, Mario, Debaix, Huguette, Festa, Beatrice Paola, Tokonami, Natsuko, Raimondi, Andrea, Cremonesi, Alessio, Carrella, Diego, Forny, Patrick, Kölker, Stefan, Diomedi Camassei, Francesca, Diaz, Francisca, Moraes, Carlos T., Di Bernardo, Diego, Baumgartner, Matthias R., Devuyst, Olivier
Format: Artigo
Idioma:Inglês
Publicat: Nature Publishing Group UK 2020
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC7033137/
https://ncbi.nlm.nih.gov/pubmed/32080200
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41467-020-14729-8
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