Sporadic Creutzfeldt-Jakob disease VM1: phenotypic and molecular characterization of a novel subtype of human prion disease

Table 3 PrP^res glycoform ratio and relative proportion of C-terminal (12–13 kDa) fragments in sCJD MM1, MV1, VM1 and VV1

sCJD subtype	MM1	MV1	VM1	VV1
N	18	9	6	7
D-PrP^res (%)	31.8 ± 2.5^a,b	29.7 ± 2.7^c	15.8 ± 3.9	22.1 ± 3.3
M-PrP^res (%)	43.6 ± 3.3^d	43.9 ± 1.1^d	45.4 ± 1.6	46.7 ± 1.9
U-PrP^res (%)	24.6 ± 3.6^a	26.3 ± 3.2^d,e	38.7 ± 3.5	31.2 ± 1.9
D/M-PrP^res ratio	1.3 ± 0.3^a,f	1.2 ± 1.1^e	0.4 ± 0.1	0.7 ± 0.1
U-CTF12-13 (%)*	19.3 ± 5.6^a	22.8 ± 8.9^c	59.3 ± 5.7	33.7 ± 9.9

Statistic comparisons (Kruskal–Wallis test): ^aversus VM1 p ≤ 0.001; ^bversus VV1 p ≤ 0.001; ^cversus VM1 p ≤ 0.05; ^dversus VV1 p ≤ 0.05; ^eversus VM1 p ≤ 0.01, ^fversus VV1 p ≤ 0.01. *relative to U-PrP^res signal
PrP^res protease-resistant core of type 1 (21 kDa) PrP^Sc, D diglycosylated isoform, M monoglycosylated isoform, U unglycosylated isoform, CTF12-13 C-terminal PrP^res fragments of 12–13 kDa

ISSN: 2051-5960