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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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Bibliografski detalji
Izdano u:eLife
Glavni autori: Yambire, King Faisal, Fernandez-Mosquera, Lorena, Steinfeld, Robert, Mühle, Christiane, Ikonen, Elina, Milosevic, Ira, Raimundo, Nuno
Format: Artigo
Jezik:Inglês
Izdano: eLife Sciences Publications, Ltd 2019
Teme:
Online pristup: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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