Transmission characteristics of heterozygous cases of Creutzfeldt-Jakob disease with variable abnormal prion protein allotypes

Table 1 PrP^Sc abundance and prion seeding activity in CJD brain homogenate samples inoculated into transgenic mice

Case #^a	Subtype^a	PrP^Sc (SpC)^b	SD₅₀/g^c
Control	MM1	ND	> 5 × 10⁹
3	MV1 + 2C	280 ± 103	5 × 10⁹
4	MV1 + 2C	48 ± 9	9 × 10⁷
9 CC	MV2K + 2C	56 ± 12	8.9 × 10¹⁰
9 CbC	MV2K + 2C	126 ± 15	2.8 × 10¹⁰
12	MV2K + 2C	90 ± 4	1.6 × 10⁹
19 (iCJD)	MV2K	133 ± 19	3.5 × 10¹¹
10	MV2K	175 ± 19	1.2 × 10¹¹
20 (iCJD)	MV2K	171 + 44	1.6 × 10¹¹
7	MV2K	581 ± 89	2.8 × 10¹¹
18 (iCJD)	MV2K	65 ± 7	2.8 × 10¹¹
11	MV2C	290 ± 9	3.4 × 10¹⁰

^aNeuropathological subtype of cases originally described in [28]. All cases are sCJD except where noted as iCJD. CC cerebral cortex, CbC cerebellar cortex
^bSpC spectral counts. Total number of PrP^Sc peptides as determined by mass spectrometry of phosphotungstic acid precipitated CJD brain material For mean + standard deviation in previous sentence, please underline the + sign. are adapted from [28]
^cSD₅₀/g = prion seeding doses (SD) yielding 50% positive wells in replicate RT-QuIC reactions per 1 g of MV brain homogenate [45]. Values were calculated from 3 independent end-point titration experiments

ISSN: 2051-5960