[HTML][HTML] Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing

D Rigamonti, S Sipione, D Goffredo, C Zuccato… - Journal of Biological …, 2001 - ASBMB
Huntington's Disease is an inherited neurodegenerative disease that affects the medium
spiny neurons in the striatum. The disease is caused by the expansion of a polyglutamine
sequence in the N terminus of Huntingtin (Htt), a widely expressed protein. Recently, we
have found that Htt is an antiapoptotic protein in striatal cells and acts by preventing caspase-
3 activity. Here we report that Htt overexpression in other CNS-derived cells can protect
them from more than 20 days exposure to fatal stimuli. In particular, we found that …