Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease

@article{Zuccato2001LossOH,
  title={Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease},
  author={C. Zuccato and A. Ciammola and D. Rigamonti and B. Leavitt and D. Goffredo and L. Conti and M. MacDonald and R. Friedlander and V. Silani and M. Hayden and T. Timmusk and S. Sipione and E. Cattaneo},
  journal={Science},
  year={2001},
  volume={293},
  pages={493 - 498}
}
Huntingtin is a 350-kilodalton protein of unknown function that is mutated in Huntington's disease (HD), a neurodegenerative disorder. The mutant protein is presumed to acquire a toxic gain of function that is detrimental to striatal neurons in the brain. However, loss of a beneficial activity of wild-type huntingtin may also cause the death of striatal neurons. Here we demonstrate that wild-type huntingtin up-regulates transcription of brain-derived neurotrophic factor (BDNF), a pro-survival… Expand
Huntington’s Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy
  • Y. Sari
  • Biology, Medicine
  • International journal of biomedical science : IJBS
  • 2011
BDNF Overexpression in the Forebrain Rescues Huntington's Disease Phenotypes in YAC128 Mice
Lack of Huntingtin-Associated Protein-1 Causes Neuronal Death Resembling Hypothalamic Degeneration in Huntington's Disease
Wild-type huntingtin up-regulates BDNF transcription in Huntington's disease
Neurotrophic factors in Huntington's disease.
Reduced expression of the TrkB receptor in Huntington's disease mouse models and in human brain
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 31 REFERENCES
Wild-Type Huntingtin Protects from Apoptosis Upstream of Caspase-3
Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells.
Inactivation of the mouse Huntington's disease gene homolog Hdh.
...
1
2
3
4
...