Expanded polyglutamine stretches form an 'aggresome'.

Journal Article (Journal Article)

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''.

Full Text

Duke Authors

Cited Authors

  • Shimohata, T; Sato, A; Burke, JR; Strittmatter, WJ; Tsuji, S; Onodera, O

Published Date

  • May 3, 2002

Published In

Volume / Issue

  • 323 / 3

Start / End Page

  • 215 - 218

PubMed ID

  • 11959423

International Standard Serial Number (ISSN)

  • 0304-3940

Digital Object Identifier (DOI)

  • 10.1016/s0304-3940(02)00162-3


  • eng

Conference Location

  • Ireland