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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...
Autores principales: | Yambire, King Faisal, Fernandez-Mosquera, Lorena, Steinfeld, Robert, Mühle, Christiane, Ikonen, Elina, Milosevic, Ira, Raimundo, Nuno |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
eLife Sciences Publications, Ltd
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6379092/ https://www.ncbi.nlm.nih.gov/pubmed/30775969 http://dx.doi.org/10.7554/eLife.39598 |
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