| First Author | Cvetanovic M | Year | 2012 |
| Journal | Neurobiol Dis | Volume | 48 |
| Issue | 3 | Pages | 526-32 |
| PubMed ID | 22884877 | Mgi Jnum | J:197504 |
| Mgi Id | MGI:5493202 | Doi | 10.1016/j.nbd.2012.07.024 |
| Citation | Cvetanovic M, et al. (2012) LANP mediates neuritic pathology in Spinocerebellar ataxia type 1. Neurobiol Dis 48(3):526-32 |
| abstractText | Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disease that results from a pathogenic glutamine-repeat expansion in the protein ataxin-1 (ATXN1). Although the functions of ATXN1 are still largely unknown, there is evidence to suggest that ATXN1 plays a role in regulating gene expression, the earliest process known to go awry in SCA1 mouse models. In this study, we show that ATXN1 reduces histone acetylation, a post-translational modification of histones associated with enhanced transcription, and represses histone acetyl transferase-mediated transcription. In addition, we find that depleting the Leucine-rich Acidic Nuclear Protein (LANP)-an ATXN1 binding inhibitor of histone acetylation-reverses aspects of SCA1 neuritic pathology. |
