Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice
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...
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| Format: | Article |
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MDPI AG
2021-09-01
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| Series: | Brain Sciences |
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| Online Access: | https://www.mdpi.com/2076-3425/11/10/1267 |
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| author | Johannes Burtscher Giuseppe Pepe Federico Marracino Luca Capocci Susy Giova Grégoire P. Millet Alba Di Pardo Vittorio Maglione |
| author_facet | Johannes Burtscher Giuseppe Pepe Federico Marracino Luca Capocci Susy Giova Grégoire P. Millet Alba Di Pardo Vittorio Maglione |
| author_sort | Johannes Burtscher |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T06:41:43Z |
| format | Article |
| id | doaj.art-a92bf5238b7f488aabdd4a42fc35ec97 |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-03-10T06:41:43Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-a92bf5238b7f488aabdd4a42fc35ec972023-11-22T17:36:30ZengMDPI AGBrain Sciences2076-34252021-09-011110126710.3390/brainsci11101267Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model MiceJohannes Burtscher0Giuseppe Pepe1Federico Marracino2Luca Capocci3Susy Giova4Grégoire P. Millet5Alba Di Pardo6Vittorio Maglione7Institute of Sport Sciences, University of Lausanne, CH-1015 Lausanne, SwitzerlandIRCCS Neuromed, 86077 Pozzilli, IS, ItalyIRCCS Neuromed, 86077 Pozzilli, IS, ItalyIRCCS Neuromed, 86077 Pozzilli, IS, ItalyIRCCS Neuromed, 86077 Pozzilli, IS, ItalyInstitute of Sport Sciences, University of Lausanne, CH-1015 Lausanne, SwitzerlandIRCCS Neuromed, 86077 Pozzilli, IS, ItalyIRCCS Neuromed, 86077 Pozzilli, IS, ItalyHuntington’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.https://www.mdpi.com/2076-3425/11/10/1267Huntington’s diseasemitochondriaoxidative phosphorylationneurodegenerationstriatumcortex |
| spellingShingle | Johannes Burtscher Giuseppe Pepe Federico Marracino Luca Capocci Susy Giova Grégoire P. Millet Alba Di Pardo Vittorio Maglione Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice Brain Sciences Huntington’s disease mitochondria oxidative phosphorylation neurodegeneration striatum cortex |
| title | Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice |
| title_full | Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice |
| title_fullStr | Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice |
| title_full_unstemmed | Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice |
| title_short | Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington’s Disease Model Mice |
| title_sort | brain region and cell compartment dependent regulation of electron transport system components in huntington s disease model mice |
| topic | Huntington’s disease mitochondria oxidative phosphorylation neurodegeneration striatum cortex |
| url | https://www.mdpi.com/2076-3425/11/10/1267 |
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