Chemical Chaperones Reduce Aggregate Formation and Cell Death Caused by the Truncated Machado–Joseph Disease Gene Product with an Expanded Polyglutamine Stretch

Chemical Chaperones Reduce Aggregate Formation and Cell Death Caused by the Truncated Machado–Joseph Disease Gene Product with an Expanded Polyglutamine Stretch

Machado–Joseph disease/spinocerebellar ataxia-3 (MJD/SCA-3) is an inherited neurodegenerative disorder caused by expansion of the polyglutamine stretch in the MJD gene-encoded protein ataxin-3. The truncated form of mutated ataxin-3 causes aggregation and cell death in vitro and in vivo. Abnormal co...

Full description

Bibliographic Details
Main Authors: Hideaki Yoshida, Toshihiro Yoshizawa, Futoshi Shibasaki, Shin'ichi Shoji, Ichiro Kanazawa
Format: Article
Language:English
Published: Elsevier 2002-07-01
Series:Neurobiology of Disease
Subjects:
Machado–Joseph disease
spinocerebellar ataxia-3
ataxin-3
polyglutamine
aggregate
cell death
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996102905024

Internet

http://www.sciencedirect.com/science/article/pii/S0969996102905024

Similar Items