Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration.
@article{Guo2013InhibitionOM, title={Inhibition of mitochondrial fragmentation diminishes Huntington's disease-associated neurodegeneration.}, author={X. Guo and M. Disatnik and Marie Monbureau and M. Shamloo and D. Mochly-Rosen and X. Qi}, journal={The Journal of clinical investigation}, year={2013}, volume={123 12}, pages={ 5371-88 } }
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… Expand
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References
SHOWING 1-10 OF 111 REFERENCES
Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli
- Biology, Medicine
- EMBO molecular medicine
- 2010
- 216
- Highly Influential
- PDF
A novel Drp1 inhibitor diminishes aberrant mitochondrial fission and neurotoxicity
- Biology, Medicine
- Journal of Cell Science
- 2013
- 240
- PDF
Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease.
- Medicine, Biology
- Human molecular genetics
- 2012
- 231
- Highly Influential
- PDF
p53 Mediates Cellular Dysfunction and Behavioral Abnormalities in Huntington’s Disease
- Biology, Medicine
- Neuron
- 2005
- 424
- Highly Influential
- PDF
Declines in Drp1 and Parkin Expression Underlie DNA Damage-Induced Changes in Mitochondrial Length and Neuronal Death
- Biology, Medicine
- The Journal of Neuroscience
- 2013
- 47
- PDF
Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease.
- Biology, Medicine
- Biochimica et biophysica acta
- 2012
- 115
Dynamin-related protein 1 and mitochondrial fragmentation in neurodegenerative diseases
- Biology, Medicine
- Brain Research Reviews
- 2011
- 232
Effects of overexpression of huntingtin proteins on mitochondrial integrity.
- Biology, Medicine
- Human molecular genetics
- 2009
- 190
- PDF
Shaping the role of mitochondria in the pathogenesis of Huntington's disease
- Biology, Medicine
- The EMBO journal
- 2012
- 121
- PDF