| Experiment Id | E-GEOD-3248 | Series Id | GSE3248 |
| Name | Transcription profiling of cerebellum from transgenic mice with nuclear and extranuclear polyQ to investigate the role of polyQ in Huntingtons disease | Experiment Type | transcription profiling by array |
| Study Type | Baseline | Source | ArrayExpress |
| Curation Date | 2019-01-08 |
| description | To dissect the impact of nuclear and extranuclear mutant htt on the initiation and progression of disease, we generated a series of transgenic mouse lines in which nuclear localization (NLS) or nuclear export sequences (NES) have been placed N-terminal to the htt exon 1 protein carrying 144 glutamines. Our data indicate that the exon 1 mutant protein is present in the nucleus as part of an oligomeric or aggregation complex. Increasing the concentration of the mutant transprotein in the nucleus is sufficient for, and dramatically accelerates the onset and progression of behavioral phenotypes. Furthermore, nuclear exon 1 mutant protein is sufficient to induce cytoplasmic neurodegeneration and transcriptional dysregulation. However, our data suggests that cytoplasmic mutant exon 1 htt, if present, contributes to disease progression.Five lines of transgenic mice and normal littermate controls were compared using Affymetrix MG-U74Av2 A arrays to examine gene expression in cerebellum. Each line had 3 or 4 replicates. Profiles were made at the first age that rotorod deficits could be detected. Some lines were also profiled at a later age when neurological impairment was more pronounced. |
