Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.

@article{Liu2009DerangedCS,
  title={Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 2.},
  author={Jing Han Liu and Tie-shan Tang and Huiping Tu and O Mancilla Nelson and Emily S Herndon and D. B. P. Huynh and Stefan Matthias Pulst and Ilya B Bezprozvanny},
  journal={The Journal of neuroscience : the official journal of the Society for Neuroscience},
  year={2009},
  volume={29 29},
  pages={
          9148-62
        }
}
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited, neurodegenerative disease caused by an expansion of polyglutamine tracts in the cytosolic protein ataxin-2 (Atx2). Cerebellar Purkinje cells (PCs) are predominantly affected in SCA2. The cause of PC degeneration in SCA2 is unknown. Here we demonstrate that mutant Atx2-58Q, but not wild-type (WT) Atx2-22Q, specifically associates with the cytosolic C-terminal region of type 1 inositol 1,4,5-trisphosphate receptor (InsP(3… CONTINUE READING
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