Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport

@article{GassetRosa2017PolyglutamineExpandedHE,
  title={Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport},
  author={F{\'a}tima Gasset-Rosa and Carlos Chill{\'o}n-Marinas and Alexander Goginashvili and Ranjit Singh Atwal and Jonathan W. Artates and Ricardos Tabet and Vanessa C. Wheeler and Anne G Bang and Don W Cleveland and Clotilde Lagier-Tourenne},
  journal={Neuron},
  year={2017},
  volume={94},
  pages={48-57.e4}
}
Onset of neurodegenerative disorders, including Huntington's disease, is strongly influenced by aging. Hallmarks of aged cells include compromised nuclear envelope integrity, impaired nucleocytoplasmic transport, and accumulation of DNA double-strand breaks. We show that mutant huntingtin markedly accelerates all of these cellular phenotypes in a dose- and age-dependent manner in cortex and striatum of mice. Huntingtin-linked polyglutamine initially accumulates in nuclei, leading to disruption… CONTINUE READING

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