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Related Experiment Videos

Huntingtin inclusions do not down-regulate specific genes in the R6/2 Huntington's disease mouse.

G Sadri-Vakili1, A S Menon, L A Farrell

  • 1Massachusetts General Hospital, MassGeneral Institute for Neurodegenerative Disease, 114 16th Street/B114- 2001, Charlestown, MA 02129-4404, USA.

The European Journal of Neuroscience
|July 6, 2006
PubMed
Summary

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Neuronal intranuclear inclusions (NIIs) in Huntington's disease (HD) do not cause gene down-regulation. This study found no difference in specific gene mRNA levels between NII-positive and NII-negative neurons in an HD mouse model.

Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Background:

  • Transcriptional dysregulation is a key factor in Huntington's disease (HD) pathogenesis.
  • Neuronal intranuclear inclusions (NIIs) are present in HD brains and R6/2 mouse models.
  • NIIs are hypothesized to contribute to transcriptional issues by sequestering transcription factors.

Purpose of the Study:

  • To investigate the relationship between NIIs and transcriptional dysregulation in the R6/2 mouse model of HD.
  • To determine if NIIs are responsible for the down-regulation of specific genes in HD.

Main Methods:

  • Utilized double-label in situ hybridization with immunohistochemistry.
  • Employed laser capture microdissection coupled with quantitative real-time PCR.
  • Compared transcript levels in NII-positive versus NII-negative neurons.

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Main Results:

  • No significant difference in D2, PPE, and PSS mRNA levels was observed between NII-positive and NII-negative neurons.
  • These findings indicate NIIs do not directly cause the down-regulation of these specific genes in the R6/2 striatum.

Conclusions:

  • NIIs are not involved in the down-regulation of specific genes investigated in this HD mouse model.
  • The results challenge the hypothesis that NIIs contribute to transcriptional dysregulation in HD.
  • Observations argue against NIIs protecting against transcriptional dysregulation in Huntington's disease.