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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...
Autores principales: | Bülow, Margret H., Wingen, Christian, Senyilmaz, Deniz, Gosejacob, Dominic, Sociale, Mariangela, Bauer, Reinhard, Schulze, Heike, Sandhoff, Konrad, Teleman, Aurelio A., Hoch, Michael, Sellin, Julia |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society for Cell Biology
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6014165/ https://www.ncbi.nlm.nih.gov/pubmed/29282281 http://dx.doi.org/10.1091/mbc.E17-08-0535 |
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