Article
Defective retrotranslocation causes loss of anti-Bax function in human familial prion protein mutants.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 9 May 2007
Jodoin Julie, Laroche-Pierre Stéphanie, Goodyer Cynthia G, LeBlanc Andréa C
Abstract excerpt
Prion protein (PrP) inhibits the activation of proapoptotic Bax in primary human neurons and MCF-7 cells. Because neuronal apoptosis occurs in human prion diseases, here we examine the anti-Bax function of familial PrP mutants. All Creutzfeldt-Jakob disease and fatal familial insomnia-associated prion protein mutations partially or completely lose the anti-Bax function in human neurons and, except for A117V and...
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