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Vol. 12, Issue 5, 1393-1407, May 2001
Max-Planck-Institut für Molekulare Genetik, D-14195 Berlin
(Dahlem), Germany
The huntingtin exon 1 proteins with a polyglutamine repeat in the
pathological range (51 or 83 glutamines), but not with a polyglutamine
tract in the normal range (20 glutamines), form aggresome-like
perinuclear inclusions in human 293 Tet-Off cells. These structures
contain aggregated, ubiquitinated huntingtin exon 1 protein with a
characteristic fibrillar morphology. Inclusion bodies with truncated
huntingtin protein are formed at centrosomes and are surrounded by
vimentin filaments. Inhibition of proteasome activity resulted in a
twofold increase in the amount of ubiquitinated, SDS-resistant
aggregates, indicating that inclusion bodies accumulate when the
capacity of the ubiquitin-proteasome system to degrade aggregation-prone huntingtin protein is exhausted. Immunofluorescence and electron microscopy with immunogold labeling revealed that the 20S,
19S, and 11S subunits of the 26S proteasome, the molecular chaperones
BiP/GRP78, Hsp70, and Hsp40, as well as the RNA-binding protein TIA-1,
the potential chaperone 14-3-3, and 
-synuclein colocalize with the
perinuclear inclusions. In 293 Tet-Off cells, inclusion body formation
also resulted in cell toxicity and dramatic ultrastructural changes
such as indentations and disruption of the nuclear envelope.
Concentration of mitochondria around the inclusions and cytoplasmic
vacuolation were also observed. Together these findings support the
hypothesis that the ATP-dependent ubiquitin-proteasome system is a
potential target for therapeutic interventions in glutamine repeat disorders.
Corresponding author. E-mail address:
wanker{at}molgen.mpg.de.
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