Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases

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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Bibliographic Details
Main Authors: King Faisal Yambire, Lorena Fernandez-Mosquera, Robert Steinfeld, Christiane Mühle, Elina Ikonen, Ira Milosevic, Nuno Raimundo
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2019-02-01
Series:eLife
Subjects:
lysosomal storage disease
mitochondria
transcriptional regulation
mitochondrial biogenesis
Online Access:https://elifesciences.org/articles/39598

Internet

https://elifesciences.org/articles/39598

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