[HTML][HTML] CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation
I Al-Ramahi, YC Lam, HK Chen, B de Gouyon… - Journal of Biological …, 2006 - ASBMB
CHIP (C terminus of Hsc-70 interacting protein) is an E3 ligase that links the protein folding
machinery with the ubiquitin-proteasome system and has been implicated in disorders
characterized by protein misfolding and aggregation. Here we investigate the role of CHIP in
protecting from ataxin-1-induced neurodegeneration. Ataxin-1 is a polyglutamine protein
whose expansion causes spinocerebellar ataxia type-1 (SCA1) and triggers the formation of
nuclear inclusions (NIs). We find that CHIP and ataxin-1 proteins directly interact and co …
machinery with the ubiquitin-proteasome system and has been implicated in disorders
characterized by protein misfolding and aggregation. Here we investigate the role of CHIP in
protecting from ataxin-1-induced neurodegeneration. Ataxin-1 is a polyglutamine protein
whose expansion causes spinocerebellar ataxia type-1 (SCA1) and triggers the formation of
nuclear inclusions (NIs). We find that CHIP and ataxin-1 proteins directly interact and co …