p80 coilin, a coiled body-specific protein, interacts with ataxin-1, the SCA1 gene product.

Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder characterized by ataxia and progressive motor deterioration. SCA1 is associated with an elongated polyglutamine tract in ataxin-1, the SCA1 gene product. Using the yeast two-hybrid system and co-immunoprecipitation experiments, we have found that p80 coilin, coiled body-specific protein, binds to ...
ataxin-1. In further experiments with deletion mutants, we found that the C-terminal regions of ataxin-1 and p80 coilin were essential for this interaction. In HeLa cells that have been co-transfected with ataxin-1 and p80 coilin, the p80 coilin protein co-localizes with ataxin-1 aggregates in the nucleoplasm. However, immunohistochemical analysis and immunofluorescence assays showed that mutant ataxin-1 aggregates do not redistribute p80 coilin's dot-like structures in the Purkinje cells of SCA1 transgenic mice. This feature of the interaction between ataxin-1 and p80 coilin suggests that p80 coilin might be implicated in altering the function of ataxin-1.
Mesh Terms:
Animals, Binding Sites, COS Cells, Hela Cells, Humans, Mice, Mice, Transgenic, Nerve Tissue Proteins, Nuclear Proteins, Protein Binding, Purkinje Cells, Recombinant Fusion Proteins, Sequence Deletion, Spinocerebellar Ataxias, Transfection, Two-Hybrid System Techniques
Biochim. Biophys. Acta
Date: May. 20, 2003
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