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  • Title: Huntingtin inclusions do not down-regulate specific genes in the R6/2 Huntington's disease mouse.
    Author: Sadri-Vakili G, Menon AS, Farrell LA, Keller-McGandy CE, Cantuti-Castelvetri I, Standaert DG, Augood SJ, Yohrling GJ, Cha JH.
    Journal: Eur J Neurosci; 2006 Jun; 23(12):3171-5. PubMed ID: 16820007.
    Abstract:
    Transcriptional dysregulation is a central pathogenic mechanism in Huntington's disease (HD); HD and transgenic mouse models of HD demonstrate down-regulation of specific genes at the level of mRNA expression. Furthermore, neuronal intranuclear inclusions (NIIs) have been identified in the brains of R6/2 mice and HD patients. One possibility is that NIIs contribute to transcriptional dysregulation by sequestering transcription factors. We therefore assessed the relationship between NIIs and transcriptional dysregulation in the R6/2 mouse, using double-label in situ hybridization combined with immunohistochemistry, and laser capture microdissection combined with quantitative real-time PCR. There was no difference in transcript levels of specific genes between NII-positive and NII-negative neurons. These results demonstrate that NIIs do not cause decreases in D2, PPE and PSS mRNA levels in R6/2 striatum and therefore are not involved in the down-regulation of these specific genes in this HD model. In addition, these observations argue against the notion that NIIs protect against transcriptional dysregulation in HD.
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