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Table 2 Transmission of Spanish and Portuguese R136S cases in TgMet129 and TgVal129 [8x] mice

From: Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease

Inocula Mice survival (attack rate) STa (n/n0)b
TgMet129 TgVal129 [8x]
Portuguese R136S case > 650 days (0/5) > 650 days (0/5)
Spanish R136S case > 650 days (0/5) > 650 days (0/5)
Sporadic CJD type I (Met129) 219 ± 17 (6/6)c 295 ± 21 (6/6)d
Sporadic CJD type II (Val129) 618 ± 81 (6/6)c 228 ± 7 (5/5)d
  1. aMean survival time
  2. bAttack rate was calculated as the relation between brain PrPres positive animals (= n) and total number of inoculated animals (= n0)
  3. cPreviously published in Cassard et al. [28], Fernández-Borges et al. [29], and Marín-Moreno et al. [30]
  4. dPreviously published in Fernández-Borges et al. [29]