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. 2024 Nov 13;14:27867. doi: 10.1038/s41598-024-79217-1

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Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 1 — Seeding lag phase and electrophoretogram of VPSPr PrPSc immunoprobed with antibody 3F4. (A) Seeding lag phases of PrPSc from non-CJD, sCJD, and VPSPr (all genotypes) 10% (w/v) brain homogenates at a 10^{− 4} dilution were seeded with recombinant full-length hamster PrPC using the real-time quaking-induced conversion (RT-QuIC) assay. Time from the assay start time to when a pre-defined amyloid formation threshold was reached (lag phase time) was calculated and plotted along with bars showing the min–max values. sCJD (n=33) displayed with a lag phase time ranging from 5 to 11 hours. VPSPr (n=20) displayed a longer lag phase, ranging from 25 to over 50 hours to reach the threshold. (B) Overlay of electrophoretograms from 29 VPSPr Cases (grey) and individual representative type 1 and 2 CJD cases (light and dark blue dotted lines, respectively) PK-digested (25 µg/ml) and immunoprobed with antibody 3F4 using the CE immunoassay.