Figure 1.

Specific reduction in ESCRT-0 proteins, Hrs and STAM1, in prion-infected mouse and human brain. A, B, Representative Western blottings and quantification of Hrs in whole-brain lysate from uninfected and terminal prion-infected mice challenged with various prion strains (n = 3–5 mice/group). *Mouse was euthanized at an early time point and not quantified (98 dpi, 55% of disease course). C, D, Representative Western blottings and quantification of ESCRT proteins from uninfected control and prion-infected (strain 22L) mice (n = 4 mice/group). ^GAPDH signal used for normalizing CHMP2B (same membrane). E, F, Representative Western blottings and quantification of Hrs in frontal cerebral cortex of unaffected or sCJD affected brain samples (n = 11 controls, n = 9 sCJD). ^#Outlier identified by Grubbs' test (excluded from graph). G, Hgs transcript levels in uninfected and prion-infected (22L) mouse brains. Relative protein levels or transcripts were normalized to the average of the unaffected mice or humans. Data shown as mean ± SEM; each dot represents an individual mouse or human; unpaired, two-tailed Student's t test, *p < 0.05, **p < 0.01, ***p < 0.001. Data supported by Extended Data Figure 1-1 and Extended Data Tables 1-1, 1-2, and 1-3.