Human Molecular Genetics, Vol 8, 673-682, Copyright © 1999 by Oxford University Press
Y Chai, SL Koppenhafer, SJ Shoesmith, MK Perez and HL Paulson
Spinocerebellar ataxia type 3, also known as Machado-Joseph disease
(SCA3/MJD), is one of at least eight inherited neurodegenerative diseases
caused by expansion of a polyglutamine tract in the disease protein. Here
we present two lines of evidence implicating the ubiquitin-proteasome
pathway in SCA3/MJD pathogenesis. First, studies of both human disease
tissue and in vitro models showed redistribution of the 26S proteasome
complex into polyglutamine aggregates. In neurons from SCA3/MJD brain, the
proteasome localized to intranuclear inclusions containing the mutant
protein, ataxin-3. In transfected cells, the proteasome redistributed into
inclusions formed by three expanded polyglutamine proteins: a pathologic
ataxin-3 fragment, full- length mutant ataxin-3 and an unrelated
GFP-polyglutamine fusion protein. Inclusion formation by the full-length
mutant ataxin-3 required nuclear localization of the protein and occurred
within specific subnuclear structures recently implicated in the regulation
of cell death, promyelocytic leukemia antigen oncogenic domains. In a
second set of experiments, inhibitors of the proteasome caused a repeat
length-dependent increase in aggregate formation, implying that the
proteasome plays a direct role in suppressing polyglutamine aggregation in
disease. These results support a central role for protein misfolding in the
pathogenesis of SCA3/MJD and suggest that modulating proteasome activity is
a potential approach to altering the progression of this and other
polyglutamine diseases.
ARTICLES
Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
Department of Neurology, University of Iowa College of Medicine, Iowa City, IA 52242, USA.
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