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
Autores principales: Yambire, King Faisal, Fernandez-Mosquera, Lorena, Steinfeld, Robert, Mühle, Christiane, Ikonen, Elina, Milosevic, Ira, Raimundo, Nuno
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2019
Materias:
Cell Biology
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

Internet

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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