Correction: Fatal Prion Disease in a Mouse Model of Genetic E200K Creutzfeldt-Jakob Disease

Abstract

[This corrects the article DOI: 10.1371/journal.ppat.1002350.].

Fig 2. Disease progression in spontaneous disease.

(A) Average disease onset and death of mice in kinetic studies. (B) Aggravation of clinical score of disease as related to the mice's age and gender. Groups of TgMHu2ME199K mice (male and female) were scored for clinical signs from birth to death. Average disease score for each group was plotted against the age elapsed since the mice birth. Closed circles: males, open circles: females. (C) Percentage of sick mice in each age group. Groups of mice (as in fig b) in which the average score was at least 1 were plotted against the age of the mice. Closed circles: males, open circles: females. (D) Relative PrP mRNA levels, as determined by quantitative RT-PCR for wt and TgMHu2ME199K/ko mice. Each bar represents the average of PrP mRNA normalized against controls genes levels (see methods) in 4 male mice. Statistical bars represent standard error (E) Brain homogenates from TgMHu2ME199K/ko, TgMHu2ME199K/wt, PrP ablated, wt C57BL/6 and RML-infected mice were immunoblotted with a PrP 6H4 mAb. (F) Relative intensities of the bands as measured by NIH Image J analysis software.

Fig 5. Biochemical Characterization of PrP in TgMHu2ME199K mice.

Fig 5. Biochemical Characterization of PrP in TgMHu2ME199K mice. (A) To establish the PrP specificity of different samples, brain homogenates from 8 months old mice from 1: TgMHu2ME199K/ko, 2: TgMHu2ME199K/wt, 3: TgMHu2M, 4: wt C57BL/6, and 5: RML infected mice, were immunoblotted with several a PrP antibodies (See Fig 3A for a PrP epitope mapping). Arrows demonstrate truncated PrP forms only present in brains of TgMHu2ME199K mice. (B) Samples as in panel a were treated in the presence or absence of PNGase and immunoblotted with designated a PrP antibodies. As above truncated PrP forms are demonstrated only in the TgMHu2ME199K mice demonstrated with arrows. (C) Samples as in panel a were extracted with sarkosyl and then centrifuged at 100000 g for 1 h, separated into pellets and supernatants. Otherwise, similar samples (denominated as total) were digested with 30 ug/ml PK for 30 min at 37uC. All samples were then immunoblotted with the designated anti PrP antibodies. Arrows demonstrate PK resistant bands in the TgMHu2ME199K samples. (D) Samples from TgMHu2ME199K/ko mice at different ages and controls were digested in the presence or absence of 30 ug/ml PK for 30 min at 37uC and immunoblotted with a PrP pAb RTC. #1: 1 month old; #2: 3 months old; #3: 7 months old; #4: another 7 months old sample, #5 PrP ablated mouse; #6 wt mouse, #7 RML infected mouse.