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Expanded polyglutamine stretches form an 'aggresome'.

Publication ,  Journal Article
Shimohata, T; Sato, A; Burke, JR; Strittmatter, WJ; Tsuji, S; Onodera, O
Published in: Neurosci Lett
May 3, 2002

To understand the pathogenetic mechanisms underlying polyglutamine (polyQ) diseases, we investigated the mechanisms of the formation of aggregate bodies containing expanded polyQ stretches, focusing on dentatorubral-pallidoluysian atrophy (DRPLA). We demonstrated that the expression of a truncated DRPLA protein containing expanded polyQ stretches in COS-7 cells resulted in the formation of perinuclear aggregate bodies that are co-localized with gamma-tubulin, a protein marker for the microtubules-organizing center (MTOC). A collapsed vimentin network surrounded these aggregate bodies. Furthermore, disruption of the microtubules (MTs) with nocodazole resulted in the formation of small aggregate bodies that were scattered throughout the cytoplasm. These findings suggest that the truncated DRPLA proteins containing expanded polyQ stretches unfold and form small aggregate bodies in the cell periphery. These aggregates move on MTs to the MTOC, where they remain as distinct 'aggresomes''.

Duke Scholars

Published In

Neurosci Lett

DOI

ISSN

0304-3940

Publication Date

May 3, 2002

Volume

323

Issue

3

Start / End Page

215 / 218

Location

Ireland

Related Subject Headings

  • Vimentin
  • Tubulin
  • Trinucleotide Repeat Expansion
  • Peptides
  • Nocodazole
  • Neurons
  • Neurodegenerative Diseases
  • Nerve Tissue Proteins
  • Myoclonic Epilepsies, Progressive
  • Microtubules
 

Citation

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Shimohata, T., Sato, A., Burke, J. R., Strittmatter, W. J., Tsuji, S., & Onodera, O. (2002). Expanded polyglutamine stretches form an 'aggresome'. Neurosci Lett, 323(3), 215–218. https://doi.org/10.1016/s0304-3940(02)00162-3
Shimohata, Takayoshi, Aki Sato, James R. Burke, Warren J. Strittmatter, Shoji Tsuji, and Osamu Onodera. “Expanded polyglutamine stretches form an 'aggresome'.Neurosci Lett 323, no. 3 (May 3, 2002): 215–18. https://doi.org/10.1016/s0304-3940(02)00162-3.
Shimohata T, Sato A, Burke JR, Strittmatter WJ, Tsuji S, Onodera O. Expanded polyglutamine stretches form an 'aggresome'. Neurosci Lett. 2002 May 3;323(3):215–8.
Shimohata, Takayoshi, et al. “Expanded polyglutamine stretches form an 'aggresome'.Neurosci Lett, vol. 323, no. 3, May 2002, pp. 215–18. Pubmed, doi:10.1016/s0304-3940(02)00162-3.
Shimohata T, Sato A, Burke JR, Strittmatter WJ, Tsuji S, Onodera O. Expanded polyglutamine stretches form an 'aggresome'. Neurosci Lett. 2002 May 3;323(3):215–218.
Journal cover image

Published In

Neurosci Lett

DOI

ISSN

0304-3940

Publication Date

May 3, 2002

Volume

323

Issue

3

Start / End Page

215 / 218

Location

Ireland

Related Subject Headings

  • Vimentin
  • Tubulin
  • Trinucleotide Repeat Expansion
  • Peptides
  • Nocodazole
  • Neurons
  • Neurodegenerative Diseases
  • Nerve Tissue Proteins
  • Myoclonic Epilepsies, Progressive
  • Microtubules