Polyglutamine domain proteins with expanded repeats bind neurofilament, altering the neurofilament network.

Journal Article (Journal Article)

Proteins with expanded polyglutamine (polyQ) repeats cause eight inherited neurodegenerative diseases. Nuclear and cytoplasmic polyQ protein is a common feature of these diseases, but its role in cell death remains debatable. Since the neuronal intermediate filament network is composed of neurofilament (NF) and NF abnormalities occur in neurodegenerative diseases, we examined whether pathologic-length polyQ domain proteins interact with NF. We expressed polyQ-green fluorescent fusion proteins (GFP) in a neuroblast cell line, TR1. Pathologic-length polyQ-GFP fusion proteins form large cytoplasmic aggregates surrounded by neurofilament. Immunoisolation of pathologic-length polyQ proteins co-isolated 68 kD NF protein demonstrating molecular interaction. These observations suggest that polyQ interaction with NF is important in the pathogenesis of the polyglutamine repeat diseases.

Full Text

Duke Authors

Cited Authors

  • Nagai, Y; Onodera, O; Strittmatter, WJ; Burke, JR

Published Date

  • 1999

Published In

Volume / Issue

  • 893 /

Start / End Page

  • 192 - 202

PubMed ID

  • 10672238

International Standard Serial Number (ISSN)

  • 0077-8923

Digital Object Identifier (DOI)

  • 10.1111/j.1749-6632.1999.tb07826.x


  • eng

Conference Location

  • United States