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Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases

Perturbations in mitochondrial function and homeostasis are pervasive in lysosomal storage diseases, but the underlying mechanisms remain unknown. Here, we report a transcriptional program that represses mitochondrial biogenesis and function in lysosomal storage diseases Niemann-Pick type C (NPC) an...

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Detalles Bibliográficos
Publicado en:eLife
Main Authors: Yambire, King Faisal, Fernandez-Mosquera, Lorena, Steinfeld, Robert, Mühle, Christiane, Ikonen, Elina, Milosevic, Ira, Raimundo, Nuno
Formato: Artigo
Idioma:Inglês
Publicado: eLife Sciences Publications, Ltd 2019
Assuntos:
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC6379092/
https://ncbi.nlm.nih.gov/pubmed/30775969
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.7554/eLife.39598
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