Journal Article Epublish
PubMed: 34679332 DOI: 10.3390/brainsci11101267
Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice.
Brain Sci 11(10)
Authors: Johannes Burtscher, Giuseppe Pepe, Federico Marracino, Luca Capocci, Susy Giova, Grégoire P Millet, Alba Di Pardo, Vittorio Maglione
Affiliations
- Institute of Sport Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland.
- Department of Biomedical Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland.
- IRCCS Neuromed, 86077 Pozzilli, IS, Italy.
Abstract
Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized by multiple metabolic dysfunctions including defects in mitochondrial homeostasis and functions. Although we have recently reported age-related changes in the respiratory capacities in different brain areas in HD mice, the precise mechanisms of how mitochondria become compromised in HD are still poorly understood. In this study, we investigated mRNA and protein levels of selected subunits of electron transport system (ETS) complexes and ATP-synthase in the cortex and striatum of symptomatic R6/2 mice. Our findings reveal a brain-region-specific differential expression of both nuclear and mitochondrial-encoded ETS components, indicating defects of transcription, translation and/or mitochondrial import of mitochondrial ETS components in R6/2 mouse brains.
MeSH
Mitochondria, Huntington Disease, Brain, Cells