We previously showed that the GSS-associated A117V PrP mutant with a valine at codon 129 retained the anti-Bax function while all of the 11 CJD-associated PrP mutations carrying a methionine at codon 129 or the FFI-associated D178N PrP mutant lost the ability to prevent Bax-mediated condensed chromatin in human neurons [31]. Here, PRNP is linked to Creutzfeldt Jacob disease.