| First Author | Jesse S | Year | 2017 |
| Journal | Sci Rep | Volume | 7 |
| Issue | 1 | Pages | 8513 |
| PubMed ID | 28819135 | Mgi Jnum | J:256566 |
| Mgi Id | MGI:6108719 | Doi | 10.1038/s41598-017-09148-7 |
| Citation | Jesse S, et al. (2017) Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington's disease. Sci Rep 7(1):8513 |
| abstractText | PGC-1alpha is a versatile inducer of mitochondrial biogenesis and responsive to the changing energy demands of the cell. As mitochondrial ATP production requires proteins that derive from translation products of cytosolic ribosomes, we asked whether PGC-1alpha directly takes part in ribosomal biogenesis. Here, we show that a fraction of cellular PGC-1alpha localizes to the nucleolus, the site of ribosomal transcription by RNA polymerase I. Upon activation PGC-1alpha associates with the ribosomal DNA and boosts recruitment of RNA polymerase I and UBF to the rDNA promoter. This induces RNA polymerase I transcription under different stress conditions in cell culture and mouse models as well as in healthy humans and is impaired already in early stages of human Huntington''s disease. This novel molecular link between ribosomal and mitochondrial biogenesis helps to explain sarcopenia and cachexia in diseases of neurodegenerative origin. |
