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Neurobiol Dis. 2012 Dec;48(3):526-32. doi: 10.1016/j.nbd.2012.07.024. Epub 2012 Aug 4.

LANP mediates neuritic pathology in Spinocerebellar ataxia type 1.

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1
Davee Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.

Abstract

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.

PMID:
22884877
PMCID:
PMC3987943
DOI:
10.1016/j.nbd.2012.07.024
[Indexed for MEDLINE]
Free PMC Article
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