Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice.
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24 septembre 2021
- doi: 10.3390/brainsci11101267
- issn: 2076-3425
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info:eu-repo/semantics/altIdentifier/doi/10.3390/brainsci11101267
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info:eu-repo/semantics/altIdentifier/pmid/34679332
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info:eu-repo/semantics/altIdentifier/pissn/2076-3425
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info:eu-repo/semantics/altIdentifier/urn/urn:nbn:ch:serval-BIB_5A2937048FB14
info:eu-repo/semantics/openAccess , CC BY 4.0 , https://creativecommons.org/licenses/by/4.0/
Mots-clés
Huntington’s disease; cortex; mitochondria; neurodegeneration; oxidative phosphorylation; striatumSujets proches
Mouse House mice Mus musculus House mouseCiter ce document
J. Burtscher et al., « Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice. », Serveur académique Lausannois, ID : 10.3390/brainsci11101267
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Résumé
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.