Fig. 6
From: Coordinate regulation of mutant NPC1 degradation by selective ER autophagy and MARCH6-dependent ERAD
FAM134B over-expression enhances degradation of I1061T NPC1. a, b Over-expression of FLAG (control vector) or FAM134B-HA (FAM-HA) in CTRL and I1061T NPC1 fibroblasts. NPC1 protein levels were quantified by a western blot and b confocal microscopy. c Over-expression of FLAG (control vector) or FAM134B lacking the LC3 interacting domain (LIR-FAM-HA) in CTRL and I1061T NPC1 fibroblasts. NPC1 was analyzed by western blot. Quantified at right. Data are mean ± s.e.m. a N = 3–6 and c N = 3 independent experiments, b >32 cells per group. a–c Student’s t-test; a (CTRL) t = 3.092, (I10) t = 3.113; b (I10) t = 5.511. n.s., not significant, *P ≤ 0.05, ****P ≤ 0.0001
