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Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling
| Content Provider | Semantic Scholar |
|---|---|
| Author | Brustovetsky, Nickolay |
| Copyright Year | 2015 |
| Abstract | Elongation of a polyglutamine (polyQ) stretch in huntingtin protein (Htt) is linked to Huntington's disease (HD) pathogenesis. The mutation in Htt correlates with neuronal dysfunction in the striatum and cerebral cortex and eventually leads to neuronal cell death. The exact mechanisms of the injurious effect of mutant Htt (mHtt) on neurons are not completely understood but might include aberrant gene transcription, defective autophagy, abnormal mitochondrial biogenesis, anomalous mitochondrial dynamics, and trafficking. In addition, deficiency in oxidative metabolism and defects in mitochondrial Ca2+ handling are considered essential contributing factors to neuronal dysfunction in HD and, consequently, in HD pathogenesis. Since the discovery of the mutation in Htt, the questions whether mHtt affects oxidative metabolism and mitochondrial Ca2+ handling and, if it does, what mechanisms could be involved were in focus of numerous investigations. However, despite significant research efforts, the detrimental effect of mHtt and the mechanisms by which mHtt might impair oxidative metabolism and mitochondrial Ca2+ handling remain elusive. In this paper, I will briefly review studies aimed at clarifying the consequences of mHtt interaction with mitochondria and discuss experimental results supporting or arguing against the mHtt effects on oxidative metabolism and mitochondrial Ca2+ handling. |
| Starting Page | 2944 |
| Ending Page | 2953 |
| Page Count | 10 |
| File Format | PDF HTM / HTML |
| DOI | 10.1007/s12035-015-9188-0 |
| PubMed reference number | 25941077 |
| Journal | Medline |
| Volume Number | 53 |
| Alternate Webpage(s) | https://scholarworks.iupui.edu/bitstream/handle/1805/15709/nihms-687441.pdf?isAllowed=y&sequence=1 |
| Alternate Webpage(s) | https://doi.org/10.1007/s12035-015-9188-0 |
| Journal | Molecular Neurobiology |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
