LANP mediates neuritic pathology in Spinocerebellar ataxia type 1

Marija Cvetanovic, Rupinder K. Kular, Puneet Opal*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

17 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)526-532
Number of pages7
JournalNeurobiology of Disease
Volume48
Issue number3
DOIs
StatePublished - Dec 2012

Keywords

  • ANP32-A
  • LANP
  • Neurite outgrowth
  • Pp32
  • SCA1
  • Spinocerebellar ataxia type 1

ASJC Scopus subject areas

  • Neurology

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