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 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.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2021 |
|---|---|
| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:11 |
|---|---|
| Enthalten in: |
Brain sciences - 11(2021), 10 vom: 24. Sept. |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Burtscher, Johannes [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
Cortex |
|---|
| Anmerkungen: |
Date Revised 26.10.2021 published: Electronic Citation Status PubMed-not-MEDLINE |
|---|
| doi: |
10.3390/brainsci11101267 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM332194744 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM332194744 | ||
| 003 | DE-627 | ||
| 005 | 20231225215108.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231225s2021 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.3390/brainsci11101267 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1107.xml |
| 035 | |a (DE-627)NLM332194744 | ||
| 035 | |a (NLM)34679332 | ||
| 035 | |a (PII)1267 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Burtscher, Johannes |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington's Disease Model Mice |
| 264 | 1 | |c 2021 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Revised 26.10.2021 | ||
| 500 | |a published: Electronic | ||
| 500 | |a Citation Status PubMed-not-MEDLINE | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Huntington’s disease | |
| 650 | 4 | |a cortex | |
| 650 | 4 | |a mitochondria | |
| 650 | 4 | |a neurodegeneration | |
| 650 | 4 | |a oxidative phosphorylation | |
| 650 | 4 | |a striatum | |
| 700 | 1 | |a Pepe, Giuseppe |e verfasserin |4 aut | |
| 700 | 1 | |a Marracino, Federico |e verfasserin |4 aut | |
| 700 | 1 | |a Capocci, Luca |e verfasserin |4 aut | |
| 700 | 1 | |a Giova, Susy |e verfasserin |4 aut | |
| 700 | 1 | |a Millet, Grégoire P |e verfasserin |4 aut | |
| 700 | 1 | |a Di Pardo, Alba |e verfasserin |4 aut | |
| 700 | 1 | |a Maglione, Vittorio |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Brain sciences |d 2011 |g 11(2021), 10 vom: 24. Sept. |w (DE-627)NLM227436393 |x 2076-3425 |7 nnns |
| 773 | 1 | 8 | |g volume:11 |g year:2021 |g number:10 |g day:24 |g month:09 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.3390/brainsci11101267 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 11 |j 2021 |e 10 |b 24 |c 09 | ||