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Exposure of A549/ABCA3−/− cells that express p.G1421R to C13 and C17 correctors increased the cleaved 180-kD ABCA3 peptide. We used IB to compare protein processing for A549/ABCA3−/− cells that express ABCA3 WT and the type I trafficking mutant p.G1421R after exposure to correctors C13 and C17. These experiments were performed twice with results similar to those of Kinting and colleagues (24). n.t. = not treated.
