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. 2022 Dec 8;7(23):e160308. doi: 10.1172/jci.insight.160308

Figure 4. Species- and mutation-specific response to DHBP.

Figure 4

(A) Primary human and mouse I1061T/I1061T or human R934L/R1077X fibroblasts were treated with 5 μM DHBP or vehicle for 5 days and analyzed by (A) Western blot for total NPC1 (quantified at right) or (B) incubated with no treatment (NT) or digested with EndoH (E) or PNGase F (P) to assess trafficking (quantified at right). Data are shown as the mean ± SEM from indicated number of independent experiments. ***P ≤ 0.001, ****P ≤ 0.0001 by t test. (A) Human I1061T, mouse I1061T, human R934L; n = 4, 4, 5; t = 7.1, 1.0, 3.8; df = 6, 6, 8; (B) Human I1061T, mouse I1061T, human R934L; n = 3, 3, 3, 3, 6, 6; t = 3.2, 5.7, 0.4, 1.0, 2.3, 1.1. df = 4, 4, 4, 4, 10, 10. See complete unedited blots in the supplemental material.