The DOI matches its Crossref record; 128 of the 128 references it cites were found in a registry or an index.
Mutant huntingtin and mitochondrial dysfunction
Open accessAbstract
Huntington's disease (HD) is a fatal inherited neurodegenerative disorder that gradually robs affected individuals of memory, cognitive skills, and normal movements. While research has identified a single faulty gene, the huntingtin gene, as the cause of the disease, a cure remains elusive. Strong evidence indicates that mitochondrial impairment plays a key role in HD pathogenesis. Here, we highlight how mtHtt might cause mitochondrial dysfunction by either perturbing transcription of nuclear-encoded mitochondrial proteins or by direct interaction with the organelle and modulation of respiration, membrane potential and Ca2+ buffering. In addition, we propose that mtHtt might convey its neurotoxicity by evoking defects in mitochondrial dynamics, organelle trafficking and fission and fusion, which in turn might result in bioenergetic failure and HD-linked neuronal dysfunction and cell death. Finally, we speculate how mitochondria may dictate selective vulnerability of long projection neurons, such as medium spiny neurons, which are particularly affected in HD.
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Oxidative Stress in Neurodegenerative Diseases: From Molecular Mechanisms to Clinical Applications
Liu, Z., Zhou, T., Ziegler, A. C., Dimitrion, P., & Zuo, L. (2017) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2017/2525967 · Cited by 810
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Johri, A., & Beal, M. F. (2012) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.112.192138 · Cited by 806
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Metadata
- Authors
- Bossy-Wetzel, E., Petrilli, A., & Knott, A. B.
- Venue
- Trends in Neurosciences
- Publisher
- Elsevier BV
- Published
- 2008-12
- Volume
- 31
- Issue
- 12
- Pages
- 609-616
- ISSN
- 0166-2236 (ISSN-L)
- OpenAlex
- W2065144533
- Cited by
- 268
- References
- 128
- FWCI
- 0.92
- Type
- journal article
- Language
- en
- Open access
- green
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
