CHAPERONE SUPPRESSION OF ATAXIN-1 AGGREGATION AND ALTERED SUBCELLULAR PROTEASOME LOCALIZATION IMPLY PROTEIN MISFOLDING IN SCA1

摘要

The present invention provides a novel method for treating neurodegenerative disease in mammals. This method involves the introduction of a therapeutic effective amount of a chaperone, a chaperone-like-compound or a compound which increases proteasome activity into the neurological system of the mammal. There is also a novel method for screening for compounds having chaperone-like activity or having activity to increase proteasome activity. The screening works in either cultured cells or animal models.