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
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
PubMed ID: 12757932
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