@inbook{3943ae0486af4aa88b14854ce5dc17e8,
title = "Regulation of ataxin-1 phosphorylation and its impact on biology",
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.",
keywords = "14-3-3, Ataxin-1, Dephosphorylation, PKA, PP2A, Phosphorylation, Polyglutamine expansion, RBM-17, SCA1, U2AF65",
author = "Sarita Lagalwar and Orr, {Harry T.}",
note = "Copyright: Copyright 2021 Elsevier B.V., All rights reserved.",
year = "2013",
doi = "10.1007/978-1-62703-411-1_13",
language = "English (US)",
isbn = "9781627034104",
series = "Methods in Molecular Biology",
publisher = "Humana Press Inc.",
pages = "201--209",
booktitle = "Trinucleotide Repeat Protocols",
}