The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Inhibition of mitochondrial protein import by mutant huntingtin
Open accessAbstract
Mitochondrial dysfunction is associated with neuronal loss in Huntington's disease (HD), a neurodegenerative disease caused by an abnormal polyglutamine expansion in huntingtin (Htt). However, the mechanisms linking mutant Htt and mitochondrial dysfunction in HD remain unknown. We identify an interaction between mutant Htt and the TIM23 mitochondrial protein import complex. Remarkably, recombinant mutant Htt directly inhibited mitochondrial protein import in vitro. Furthermore, mitochondria from brain synaptosomes of presymptomatic HD model mice and from mutant Htt-expressing primary neurons exhibited a protein import defect, suggesting that deficient protein import is an early event in HD. The mutant Htt–induced mitochondrial import defect and subsequent neuronal death were attenuated by overexpression of TIM23 complex subunits, demonstrating that deficient mitochondrial protein import causes mutant Htt-induced neuronal death. Collectively, these findings provide evidence for a direct link between mutant Htt, mitochondrial dysfunction and neuronal pathology, with implications for mitochondrial protein import–based therapies in HD.
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Cites
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Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines
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Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities
Tabrizi, S. J., Flower, M. D., Ross, C. A., & Wild, E. J. (2020) · Nature Reviews Neurology · doi:10.1038/s41582-020-0389-4 · Cited by 538
Huntington’s Disease: Mechanisms of Pathogenesis and Therapeutic Strategies
Jimenez-Sanchez, M., Licitra, F., Underwood, B. R., & Rubinsztein, D. C. (2017) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a024240 · Cited by 507
Mitochondrial dysfunction in neurodegenerative diseases and the potential countermeasure
Wang, Y., Xu, E., Musich, P. R., & Lin, F. (2019) · CNS Neuroscience & Therapeutics · doi:10.1111/cns.13116 · Cited by 294
Energy matters: presynaptic metabolism and the maintenance of synaptic transmission
Li, S., & Sheng, Z.-H. (2022) · Nature Reviews Neuroscience · doi:10.1038/s41583-021-00535-8 · Cited by 267
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Cai, Q., & Jeong, Y. Y. (2020) · Cells · doi:10.3390/cells9010150 · Cited by 265
Metadata
- Authors
- Yano, H., Baranov, S. V., Baranova, O. V., Kim, J., Pan, Y., Yablonska, S., Carlisle, D. L., Ferrante, R. J., Kim, A. H., & Friedlander, R. M.
- Venue
- Nature Neuroscience
- Published
- 2014-05-18
- Volume
- 17
- Issue
- 6
- Pages
- 822-831
- ISSN
- 1097-6256 (ISSN-L)
- 1546-1726
- OpenAlex
- W2101699102
- Cited by
- 227
- References
- 8
- FWCI
- 8.05
- Type
- journal article
- Language
- en
- Open access
- green
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- Yano, H., Baranov, S. V., Baranova, O. V., Kim, J., Pan, Y., Yablonska, S., Carlisle, D. L., Ferrante, R. J., Kim, A. H., & Friedlander, R. M. (2014). Inhibition of mitochondrial protein import by mutant huntingtin. Nature Neuroscience. https://doi.org/10.1038/nn.3721
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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).
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