Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice.

Details

Serval ID
serval:BIB_5A2937048FB1
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice.
Journal
Brain sciences
Author(s)
Burtscher J., Pepe G., Marracino F., Capocci L., Giova S., Millet G.P., Di Pardo A., Maglione V.
ISSN
2076-3425 (Print)
ISSN-L
2076-3425
Publication state
Published
Issued date
24/09/2021
Peer-reviewed
Oui
Volume
11
Number
10
Pages
1267
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
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.
Keywords
Huntington’s disease, cortex, mitochondria, neurodegeneration, oxidative phosphorylation, striatum
Pubmed
Open Access
Yes
Create date
08/11/2021 10:48
Last modification date
24/02/2022 7:34
Usage data