Protein Misfolding and Cellular Stress in Disease and Aging

Volume 648 of the series Methods in Molecular Biology pp 215-229


Transcription Factor Sequestration by Polyglutamine Proteins

  • Tomoyuki YamanakaAffiliated withLaboratory for Structural Neuropathology, RIKEN Brain Science InstituteSpecial Postdoctoral Researchers Program, RIKEN
  • , Nobuyuki NukinaAffiliated withLaboratory for Structural Neuropathology, RIKEN Brain Science Institute Email author 

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In polyglutamine diseases including Huntington’s disease, the causative gene products containing expanded polyglutamine form nuclear aggregates in neurons. Recent studies have identified several transcriptional factors, which interact with and are sequestered by expanded polyglutamine aggregates in neurons. Further, altered expression of many genes has been shown in several polyglutamine disease models. These observations suggest an involvement of transcriptional dysregulation in pathological process of these diseases. In this chapter, we introduce several methods to examine the interaction of transcriptional factors with and their sequestration by expanded polyglutamine proteins in vitro and in vivo.

Key words

Polyglutamine Huntington’s disease Huntingtin Transcriptional factor Sequestration Aggregate Western blot analysis Filter trap assay Immunofluorescence microscopy EMSA