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. 2022 Mar 25;14:848991. doi: 10.3389/fnagi.2022.848991

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Copyright © 2022 Cazzaniga, Bistaffa, De Luca, Portaleone, Catania, Redaelli, Tramacere, Bufano, Rossi, Caroppo, Giovagnoli, Tiraboschi, Di Fede, Eleopra, Devigili, Elia, Cilia, Fiorini, Bongianni, Salzano, Celauro, Quarta, Mammana, Legname, Tagliavini, Parchi, Zanusso, Giaccone and Moda.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Analysis of the biochemical properties of PrP^res generated at each round of amplification from BH and OM of MM1, MV2 and VV2 patients. From the first appearance to the end of the amplification the PrP^res maintained both glycoform ratio and typing. (A) The BH and OM of patient 3 generated type 2 PrP^res with a predominant di-glycosylated species; (B) BH and OM of patients 12 generated type 2 and type 1 PrP^res, respectively, that were also characterized by distinct glycoform ratios; (C) OM of patient 7 remained negative while BH gave rise to type 1 PrP^res with an equal representation of the di- and mono-glycosylated species; (D) BH and OM of patient 16 generated type 1 PrP^res with different glycoform ratios. (E) BH and OM of patient 26 generated type 1 PrP^res with similar levels of the di- and mono-glycosylated isoforms.