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

Brain Sciences

Luca Capocci¹  Alba Di Pardo¹  Vittorio Maglione¹  Susy Giova¹  Federico Marracino¹  Giuseppe Pepe¹  Johannes Burtscher²  Grégoire P. Millet² 

[1] IRCCS Neuromed, 86077 Pozzilli, IS, Italy;Institute of Sport Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland;

关键词: Huntington’s disease;    mitochondria;    oxidative phosphorylation;    neurodegeneration;    striatum;    cortex;   

DOI  :  10.3390/brainsci11101267

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown