[HTML][HTML] Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes

NR Jana, P Dikshit, A Goswami, S Kotliarova… - Journal of Biological …, 2005 - ASBMB
A major hallmark of the polyglutamine diseases is the formation of neuronal intranuclear
inclusions of the disease proteins that are ubiquitinated and often associated with various
chaperones and proteasome components. But, how the polyglutamine proteins are
ubiquitinated and degraded by the proteasomes are not known. Here, we demonstrate that
CHIP (C terminus of Hsp70-interacting protein) co-immunoprecipitates with the
polyglutamine-expanded huntingtin or ataxin-3 and associates with their aggregates …