Impaired ganglioside metabolism in Huntington's disease and neuroprotective role of GM1.

@article{Maglione2010ImpairedGM,
  title={Impaired ganglioside metabolism in Huntington's disease and neuroprotective role of GM1.},
  author={Vittorio Maglione and Paolo Marchi and Alba Di Pardo and Susanne Lingrell and Melanie Horkey and Emily Tidmarsh and Simonetta Sipione},
  journal={The Journal of neuroscience : the official journal of the Society for Neuroscience},
  year={2010},
  volume={30 11},
  pages={
          4072-80
        }
}
Huntington's disease (HD) is a neurodegenerative disorder caused by the expansion of a polyglutamine stretch in the protein huntingtin (Htt). HD neurons are dysfunctional at multiple levels and have increased susceptibility to stress and apoptotic stimuli. We have discovered that synthesis of the ganglioside GM1 is reduced in fibroblasts from HD patients and in cell and animal models of HD, and that decreased GM1 levels contribute to heighten HD cell susceptibility to apoptosis. The apoptotic… CONTINUE READING

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