Prion protein: Difference between revisions
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==Hot Spots in PrP<sup>C</sup> for pathogenic conversion== | ==Hot Spots in PrP<sup>C</sup> for pathogenic conversion== | ||
There are several point mutations associated with known human prion diseases (P102L, P105L, A117V, M129V, G131V, Y145Stop, R148H, Q160Stop, D178N, V180I, T183A, H187R, T188R, E196K, F198S, E200K, D202N, V203I, R208H, V210I, E211Q, Q212P, and Q217R). | There are several <scene name='Prion_protein/Prion_point_mutations/1'>point mutations associated with known human prion diseases</scene> (P102L, P105L, A117V, M129V, G131V, Y145Stop, R148H, Q160Stop, D178N, V180I, T183A, H187R, T188R, E196K, F198S, E200K, D202N, V203I, R208H, V210I, E211Q, Q212P, and Q217R). | ||
The pathogenic conversion process from PrP<sup>C</sup> to PrP<sup>Sc</sup> could be related to the thermal stability of PrP<sup>C</sup> <ref>Kuwata, K. ''et al.'' (2007) Hot spots in prion protein for pathogenic conversion ''Proc. Natl. Acad. Sci. USA'' '''104''', 11921–11926</ref>, since the mutations related to familial forms of the prion diseases are rather concentrated in helices 2 and 3, and the thermodynamical stability profile shows that diverse residues in helices 2 and 3 are less stable <ref>Kuwata, K. ''et al.'' (2002) Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrP<sup>Sc</sup> ''Biochemistry '' '''41''', 12277–12283</ref>. | The pathogenic conversion process from PrP<sup>C</sup> to PrP<sup>Sc</sup> could be related to the thermal stability of PrP<sup>C</sup> <ref>Kuwata, K. ''et al.'' (2007) Hot spots in prion protein for pathogenic conversion ''Proc. Natl. Acad. Sci. USA'' '''104''', 11921–11926</ref>, since the mutations related to familial forms of the prion diseases are rather concentrated in helices 2 and 3, and the thermodynamical stability profile shows that diverse residues in helices 2 and 3 are less stable <ref>Kuwata, K. ''et al.'' (2002) Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrP<sup>Sc</sup> ''Biochemistry '' '''41''', 12277–12283</ref>. | ||
Moreover, the conversion might also be related with the global conformational fluctuation of PrP<sup>C</sup>, as a Carr–Purcell–Meiboom–Gill relaxation–dispersion | Moreover, the conversion might also be related with the global conformational fluctuation of PrP<sup>C</sup>, as a Carr–Purcell–Meiboom–Gill relaxation–dispersion | ||