Pambianco 2016 Cell Rep: Difference between revisions
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Copyright ยฉ 2016 The Authors. Published by Elsevier Inc. All rights reserved. | Copyright ยฉ 2016 The Authors. Published by Elsevier Inc. All rights reserved. | ||
|mipnetlab=IT Milan Clementi E | |||
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Revision as of 12:40, 20 January 2017
| Pambianco S, Giovarelli M, Perrotta C, Zecchini S, Cervia D3, Di Renzo I, Moscheni C, Ripolone M, Violano R, Moggio M, Bassi MT, Puri PL, Latella L, Clementi E, De Palma C (2016) Reversal of defective mitochondrial biogenesis in limb-girdle muscular dystrophy 2D by independent modulation of histone and PGC-1ฮฑ acetylation. Cell Rep 17:3010-23. |
Pambianco S, Giovarelli M, Perrotta C, Zecchini S, Cervia D3, Di Renzo I, Moscheni C, Ripolone M, Violano R, Moggio M, Bassi MT, Puri PL, Latella L, Clementi E, De Palma C (2016) Cell Rep
Abstract: Mitochondrial dysfunction occurs in many muscle degenerative disorders. Here, we demonstrate that mitochondrial biogenesis was impaired in limb-girdle muscular dystrophy (LGMD) 2D patients and mice and was associated with impaired OxPhos capacity. Two distinct approaches that modulated histones or peroxisome proliferator-activated receptor-gamma coactivator 1 ฮฑ (PGC-1ฮฑ) acetylation exerted equivalent functional effects by targeting different mitochondrial pathways (mitochondrial biogenesis or fatty acid oxidation[FAO]). The histone deacetylase inhibitor Trichostatin A (TSA) changed chromatin assembly at the PGC-1ฮฑ promoter, restored mitochondrial biogenesis, and enhanced muscle oxidative capacity. Conversely, nitric oxide (NO) triggered post translation modifications of PGC-1ฮฑ and induced FAO, recovering the bioenergetics impairment of muscles but shunting the defective mitochondrial biogenesis. In conclusion, a transcriptional blockade of mitochondrial biogenesis occurred in LGMD-2D and could be recovered by TSA changing chromatin conformation, or it could be overcome by NO activating a mitochondrial salvage pathway.
Copyright ยฉ 2016 The Authors. Published by Elsevier Inc. All rights reserved.
โข O2k-Network Lab: IT Milan Clementi E
Labels: MiParea: Respiration, mt-Biogenesis;mt-density, mtDNA;mt-genetics
Pathology: Other
Organism: Mouse Tissue;cell: Skeletal muscle Preparation: Intact cells, Permeabilized cells, Isolated mitochondria
Coupling state: LEAK, OXPHOS
Pathway: F, N, S, NS, ROX
HRR: Oxygraph-2k
Labels, 2017-01
