| First Author | Euler P | Year | 2012 |
| Journal | Neurosci Lett | Volume | 517 |
| Issue | 1 | Pages | 7-12 |
| PubMed ID | 22712074 | Mgi Jnum | J:333611 |
| Mgi Id | MGI:6872559 | Doi | 10.1016/j.neulet.2012.03.080 |
| Citation | Euler P, et al. (2012) Gene expression analysis on a single cell level in Purkinje cells of Huntington's disease transgenic mice. Neurosci Lett 517(1):7-12 |
| abstractText | Ataxia is a clinical feature of most polyglutamine disorders. Cerebellar neurodegeneration of Purkinje cells (PCs) in Huntington's Disease (HD) brain was described in the 1980s. PC death in the R6/2 transgenic model for HD was published by Turmaine et al. So far, PCs have not been examined on a single cell level. In order to begin to understand PC dysfunction and degeneration in HD we performed a gene expression study on laser-dissected PC based on a DNA microarray screening and quantitative real time PCR (Q-PCR). We demonstrate downregulation of the retinoid acid receptor-related orphan receptor (ROR) mRNA and ROR-mediated mRNAs, also seen by immunofluorescent staining. As ROR and ROR-dependent transcriptional dysregulation is not only found in the R6/2 model for HD but also in a model for spinocerebellar ataxia type 1 (SCA1) (Serra et al.) the data suggest common pathogenic mechanisms for both polyglutamine diseases. |
