Skip to main content
Journal cover image

Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network.

Publication ,  Journal Article
Nagai, Y; Onodera, O; Chun, J; Strittmatter, WJ; Burke, JR
Published in: Exp Neurol
February 1999

Eight inherited neurodegenerative diseases are caused by genes with expanded CAG repeats coding for polyglutamine domains in the disease-producing proteins. The mechanism by which this expanded polyglutamine domain causes neurodegenerative disease is unknown, but nuclear and cytoplasmic polyglutamine protein aggregation is a common feature. In transfected COS7 cells, expanded polyglutamine proteins aggregate and disrupt the vimentin intermediate filament network. Since neurons have an intermediate filament network composed of neurofilament (NF) and NF abnormalities occur in neurodegenerative diseases, we examined whether pathologic-length polyglutamine domain proteins also interact with NF. We expressed varying lengths polyglutamine-green fluorescent protein fusion proteins in a neuroblast cell line, TR1. Pathologic-length polyglutamine-GFP fusion proteins formed large cytoplasmic aggregates surrounded by neurofilament. Immunoisolation of pathologic-length polyglutamine proteins coisolated 68-kDa NF protein demonstrating molecular interaction. These observations suggest that polyglutamine interaction with NF is important in the pathogenesis of the polyglutamine repeat diseases.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

Exp Neurol

DOI

ISSN

0014-4886

Publication Date

February 1999

Volume

155

Issue

2

Start / End Page

195 / 203

Location

United States

Related Subject Headings

  • Tumor Cells, Cultured
  • Transfection
  • Repetitive Sequences, Nucleic Acid
  • Recombinant Fusion Proteins
  • Protein Binding
  • Peptides
  • Neurons
  • Neurology & Neurosurgery
  • Neurofilament Proteins
  • Neurodegenerative Diseases
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Nagai, Y., Onodera, O., Chun, J., Strittmatter, W. J., & Burke, J. R. (1999). Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network. Exp Neurol, 155(2), 195–203. https://doi.org/10.1006/exnr.1998.6991
Nagai, Y., O. Onodera, J. Chun, W. J. Strittmatter, and J. R. Burke. “Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network.Exp Neurol 155, no. 2 (February 1999): 195–203. https://doi.org/10.1006/exnr.1998.6991.
Nagai Y, Onodera O, Chun J, Strittmatter WJ, Burke JR. Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network. Exp Neurol. 1999 Feb;155(2):195–203.
Nagai, Y., et al. “Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network.Exp Neurol, vol. 155, no. 2, Feb. 1999, pp. 195–203. Pubmed, doi:10.1006/exnr.1998.6991.
Nagai Y, Onodera O, Chun J, Strittmatter WJ, Burke JR. Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network. Exp Neurol. 1999 Feb;155(2):195–203.
Journal cover image

Published In

Exp Neurol

DOI

ISSN

0014-4886

Publication Date

February 1999

Volume

155

Issue

2

Start / End Page

195 / 203

Location

United States

Related Subject Headings

  • Tumor Cells, Cultured
  • Transfection
  • Repetitive Sequences, Nucleic Acid
  • Recombinant Fusion Proteins
  • Protein Binding
  • Peptides
  • Neurons
  • Neurology & Neurosurgery
  • Neurofilament Proteins
  • Neurodegenerative Diseases