Figure 5.

Phenotypic effects of selective UCHL1 inhibition in idiopathic pulmonary fibrosis (IPF). (a) Schematic of TGF-β1-mediated fibroblast-to-myofibroblast transition in primary human lung fibroblasts, increasing alpha-smooth muscle actin (αSMA) transdifferentiation marker. (b) Primary fibroblasts from IPF donors were preincubated with 1 μM 1, 2, 3, LDN-57444, or IPF approved drug (nintedanib) for 1 h followed by TGF-β1 treatment for 3 days. αSMA expression was analyzed by high content imaging, demonstrating 1, 2, and nintedanib, but not 3 or LDN-57444, inhibit transdifferentiation (N.S. nonsignificant, *P ≤ 0.05, ***P ≤ 0.01). Plots represent median values (center lines) and 25th/75th percentiles (box limits) with Tukey whiskers.