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J Biol Chem, Vol. 275, Issue 12, 8772-8778, March 24, 2000
From the Spinal and bulbar muscular atrophy (SBMA) is one
of a group of human inherited neurodegenerative diseases caused by
polyglutamine expansion. We have previously demonstrated that the SBMA
gene product, the androgen receptor protein, is toxic and aggregates when truncated. Heat shock proteins function as molecular chaperones, which recognize and renaturate misfolded protein (aggregate). We thus
assessed the effect of a variety of chaperones in a cultured neuronal
cell model of SBMA. Overexpression of chaperones reduces aggregate
formation and suppresses apoptosis in a cultured neuronal cell model of
SBMA to differing degrees depending on the chaperones and their
combinations. Combination of Hsp70 and Hsp40 was the most effective
among the chaperones in reducing aggregate formation and providing
cellular protection, reflecting that Hsp70 and Hsp40 act together in
chaperoning mutant and disabled proteins. Although Hdj2/Hsdj chaperone
has been previously reported to suppress expanded polyglutamine
tract-formed aggregate, Hsdj/Hdj2 showed little effect in our system.
These findings indicate that chaperones may be one of the key factors
in the developing of CAG repeat disease and suggested that increasing
expression level or enhancing the function of chaperones will provide
an avenue for the treatment of CAG repeat disease.
Chaperones Hsp70 and Hsp40 Suppress Aggregate Formation and
Apoptosis in Cultured Neuronal Cells Expressing Truncated Androgen
Receptor Protein with Expanded Polyglutamine Tract*
,,,,¶
Department of Neurology, Nagoya
University School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya
466-8550, Japan and the § Laboratory of Experimental Radiology, Aichi
Cancer Center Research Institute, Chikusa-ku,
Nagoya 464-8681, Japan
*
This work was supported by grants from the Ministry of
Health and Welfare of Japan, by a Center of Excellence grant from the Ministry of Education, Sciences, Sports and Culture of Japan, and by a
grant from the Naitou foundation.The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
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|
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|
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|
|
|
|
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|
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|
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|
|
|
|
|
|
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|
|
|
|
|
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|
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