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- Title
Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model.
- Authors
Watase, Kei; Gatchel, Jennifer R; Sun, Yaling; Emamian, Effat; Atkinson, Richard; Richman, Ronald; Mizusawa, Hidehiro; Orr, Harry T; Shaw, Chad; Zoghbi, Huda Y
- Abstract
Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disorder characterized by progressive motor and cognitive dysfunction. Caused by an expanded polyglutamine tract in ataxin 1 (ATXN1), SCA1 pathogenesis involves a multifactorial process that likely begins with misfolding of ATXN1, which has functional consequences on its interactions, leading to transcriptional dysregulation. Because lithium has been shown to exert neuroprotective effects in a variety of conditions, possibly by affecting gene expression, we tested the efficacy of lithium treatment in a knock-in mouse model of SCA1 (Sca1(154Q/2Q) mice) that replicates many features of the human disease.
- Publication
PLoS medicine, 2007, Vol 4, Issue 5, pe182
- ISSN
1549-1676
- Publication type
Journal Article
- DOI
10.1371/journal.pmed.0040182