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Stressing a role for Staufen1 in SCA2 pathogenesis

Nature Reviews Neurology volume 14, page 634 (2018)Cite this article

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A new report in Nature Communications has identified a key role for the RNA-binding protein Staufen1 (STAU1) in the pathogenesis of spinocerebellar ataxia type 2 (SCA2), a form of cerebellar ataxia caused by CAG repeat expansions in the ataxin 2 gene (ATXN2). STAU1 was found to contribute to dysregulation of RNA metabolism through an interaction with the ATXN2 protein.

Pathologically, SCA2 is characterized by accumulation of ATXN2 in stress granules, which are aggregates of RNA and protein that form under conditions of cellular stress and consist largely of stalled mRNA translation initiation complexes. Pulst and colleagues observed colocalization of STAU1 and ATXN2 in stress granules in fibroblasts from patients with SCA2 and in cerebellar Purkinje cells in an SCA2 mouse model.

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References

Original article

  • Paul, S. et al. Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration. Nat. Commun. 9, 3648 (2018)

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  1. Nature Reviews Neurology http://www.nature.com/nrneurol/

    Heather Wood

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  1. Heather Wood
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Correspondence to Heather Wood.

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Wood, H. Stressing a role for Staufen1 in SCA2 pathogenesis. Nat Rev Neurol 14, 634 (2018). https://doi.org/10.1038/s41582-018-0080-1

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