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Unbalanced lipolysis results in lipotoxicity and mitochondrial damage in peroxisome-deficient Pex19 mutants

Inherited peroxisomal biogenesis disorders (PBDs) are characterized by the absence of functional peroxisomes. They are caused by mutations of peroxisomal biogenesis factors encoded by Pex genes, and result in childhood lethality. Owing to the many metabolic functions fulfilled by peroxisomes, PBD pa...

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Detalhes bibliográficos
Publicado no:Mol Biol Cell
Main Authors: Bülow, Margret H., Wingen, Christian, Senyilmaz, Deniz, Gosejacob, Dominic, Sociale, Mariangela, Bauer, Reinhard, Schulze, Heike, Sandhoff, Konrad, Teleman, Aurelio A., Hoch, Michael, Sellin, Julia
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society for Cell Biology 2018
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6014165/
https://ncbi.nlm.nih.gov/pubmed/29282281
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1091/mbc.E17-08-0535
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