Fig. 8.

Flux through ER-phagy is altered in Niemann–Pick C mouse and patient brain. a Whole brain homogenates (Hom) from 7-week-old WT and I1061T-Npc1 (I10) mice, or fractions enriched for cytosol (Cyt), ER, autophagosomes (APG), autolysosomes (AUT), or lysosomes (Lys) were analyzed by western blot. Blots were probed for FAM134B. Quantified at right relative to total protein as determined by Ponceau S stain (see Supplementary Fig. 8a, b). b Relative FAM134B mRNA levels in the brain of 7-week-old WT and I1061T-Npc1 mice. c Cerebellar lysates from control (CTRL1, CTRL2) and Niemann–Pick C (NP1, NP2) subjects were analyzed by western blot. d Model of I1061T degradation by ERAD and ER-phagy. Data are mean ± s.e.m. from a, b N = 3 independent experiments. a ANOVA with Tukey’s post-hoc, F = 3.85, b Student’s t-test, n.s. = not significant, **P ≤ 0.01