Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.

@article{Kim2013StructuralBO,
  title={Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.},
  author={E. Kim and Hsiang-Chih Lu and H. Zoghbi and J. Song},
  journal={Genes & development},
  year={2013},
  volume={27 6},
  pages={
          590-5
        }
}
Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease caused by polyglutamine expansion in Ataxin-1 (ATXN1). ATXN1 binds to the transcriptional repressor Capicua (CIC), and the interaction plays a critical role in SCA1 pathogenesis whereby reducing CIC levels rescues SCA1-like phenotypes in a mouse model. The ATXN1/HBP1 (AXH) domain of ATXN1 mediates its homodimerization as well as the interaction with CIC. Here, we present the crystal structure of ATXN1's AXH… Expand
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