Regulation of ataxin-1 phosphorylation and its impact on biology

Sarita Lagalwar, Harry T. Orr

Research output: Chapter in Book/Report/Conference proceedingChapter

9 Scopus citations

Abstract

Ataxin-1 protein expression is found in the cytoplasm and nucleus of Purkinje cells, the primary site of spinocerebellar ataxia type 1 (SCA1). Phosphorylation at S776 occurs in the cytoplasm and stabilizes the protein through interaction with 14-3-3, allowing it to translocate into the nucleus where disease is initiated. Phosphorylation and stabilization are enhanced when the polyglutamine expansion is present. In this chapter, we present a model of neurodegeneration in SCA1 initiated through phosphorylation at S776 by cAMP-dependent protein kinase (PKA) and enhanced by the presence of the polyglutamine expansion. The biological methods used to uncover SCA1 pathogenesis and phosphorylation at S776 are described.

Original languageEnglish (US)
Title of host publicationTrinucleotide Repeat Protocols
PublisherHumana Press Inc.
Pages201-209
Number of pages9
ISBN (Print)9781627034104
DOIs
StatePublished - 2013

Publication series

NameMethods in Molecular Biology
Volume1010
ISSN (Print)1064-3745

Keywords

  • 14-3-3
  • Ataxin-1
  • Dephosphorylation
  • PKA
  • PP2A
  • Phosphorylation
  • Polyglutamine expansion
  • RBM-17
  • SCA1
  • U2AF65

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