The DOI matches its Crossref record; 15 of the 15 references it cites were found in a registry or an index.
Inhibition of mitochondrial fragmentation diminishes Huntington’s disease–associated neurodegeneration
Open accessAbstract
Huntington's disease (HD) is the result of expression of a mutated Huntingtin protein (mtHtt), and is associated with a variety of cellular dysfunctions including excessive mitochondrial fission. Here, we tested whether inhibition of excessive mitochondrial fission prevents mtHtt-induced pathology. We developed a selective inhibitor (P110-TAT) of the mitochondrial fission protein dynamin-related protein 1 (DRP1). We found that P110-TAT inhibited mtHtt-induced excessive mitochondrial fragmentation, improved mitochondrial function, and increased cell viability in HD cell culture models. P110-TAT treatment of fibroblasts from patients with HD and patients with HD with iPS cell-derived neurons reduced mitochondrial fragmentation and corrected mitochondrial dysfunction. P110-TAT treatment also reduced the extent of neurite shortening and cell death in iPS cell-derived neurons in patients with HD. Moreover, treatment of HD transgenic mice with P110-TAT reduced mitochondrial dysfunction, motor deficits, neuropathology, and mortality. We found that p53, a stress gene involved in HD pathogenesis, binds to DRP1 and mediates DRP1-induced mitochondrial and neuronal damage. Furthermore, P110-TAT treatment suppressed mtHtt-induced association of p53 with mitochondria in multiple HD models. These data indicate that inhibition of DRP1-dependent excessive mitochondrial fission with a P110-TAT-like inhibitor may prevent or slow the progression of HD.
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p53 Regulates Mitochondrial Respiration
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The cell biology of mitochondrial membrane dynamics
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Mitochondrial Dynamics and Its Involvement in Disease
Chan, D. C. (2020) · Annual Review of Pathology: Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012419-032711 · Cited by 1,416
Mitochondrial dynamics in health and disease: mechanisms and potential targets
Chen, W., Zhao, H., & Li, Y. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01547-9 · Cited by 1,306
Distinct fission signatures predict mitochondrial degradation or biogenesis.
Kleele, T., Rey, T., Winter, J., Zaganelli, S., Mahecic, D., Perreten Lambert, H., Ruberto, F. P., Nemir, M., Wai, T., Pedrazzini, T., et al. (2021) · Nature · doi:10.1038/s41586-021-03510-6 · Cited by 794
Pluripotent stem cells in disease modelling and drug discovery
Avior, Y., Sagi, I., & Benvenisty, N. (2016) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm.2015.27 · Cited by 592
Metadata
- Authors
- Guo, X., Disatnik, M.-H., Monbureau, M., Shamloo, M., Mochly-Rosen, D., & Qi, X.
- Venue
- Journal of Clinical Investigation
- Published
- 2013-11-15
- Volume
- 123
- Issue
- 12
- Pages
- 5371-5388
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W2010369463
- Cited by
- 370
- References
- 15
- FWCI
- 12.38
- Type
- journal article
- Language
- en
- Open access
- green
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- Guo, X., Disatnik, M.-H., Monbureau, M., Shamloo, M., Mochly-Rosen, D., & Qi, X. (2013). Inhibition of mitochondrial fragmentation diminishes Huntington’s disease–associated neurodegeneration. Journal of Clinical Investigation. https://doi.org/10.1172/jci70911
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