Figure 13. Model of PrP 57-231 showing the binding sites of Cu2+ ions. (Reproduced by permission from [49]).

This domain contains several His residues [52], which may act as anchoring sites to bind Cu2+ ions (Fig. 14) [31]. There are some indications that a pep-tide corresponding to the second helical region of the human PrP shows some ability to form fibrils and it possesses a Cu2+-mediated neurotoxicity [53]. The abnormal interactions with model membranes may also have some impact on the neurotoxicity of this fragment [54].

His187 acts as an anchoring site for Cu2+ ions and the binding motif seems to be similar to those found in the other fragments of mammalian PrP [55].

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