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. 2021 Jul 29;142(4):707–728. doi: 10.1007/s00401-021-02350-y

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© The Author(s) 2021

Open AccessThis 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 — Immunoblot profile of PrP^Sc in genetic CJD. a Genetic CJD haplotypes associated with PrP^Sc type 1 (lanes 3, 5) and “i” (lane 6). As in sCJD MM1, monoglycosylated PrP^Sc was predominant in the V210I mutation, while the diglycosylated was the most represented isoform in patients carrying the E200K variant. D178N-129V carriers showed an equal mix of mono- and diglycosylated isoforms, whereas the unglycosylated band was underrepresented. b Genetic CJD haplotypes associated with PrP^Sc type 2 (lanes 2, 3, 5 and 7) and 1 + 2 (lane 6). The unglycosylated isoform in a 5-OPRI-129V case with heterozygosity (MV) at codon 129 (lane 5) is characterized by a doublet migrating at 19 and 20 kDa (PrP^Sc type 2 + “i”). The T183A mutation showed a profile characterized by a marked under-representation of the diglycosylated isoform as compared with the monoglycosylated band. c Immunoblot profile of D178N-129V cases. The unglycosylated isoform is represented by doublet with variable dominance of either the band migrating at 21kDa (PrP^Sc type 1) or the one migrating slightly faster. A similar pattern of migration was observed in sCJD VV1 (lane 1). a2 and c3 lanes showed the same case. Samples were resolved in 7 (a, b) and 15 cm (c) long gels and probed with the primary antibody 3F4