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Figure 1 | Orphanet Journal of Rare Diseases

Figure 1

From: Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines

Figure 1

Some F508del-CFTR correctors correct the trafficking of hERG G601S. Representative immunoblots are shown for decreasing concentrations of ABT-888 and glafenine (A), ouabain (B), sildenafil, KM60, and KM57 (C), RDR1 (D), telenzepine and latonduine (E). In all blots, negative control (DMSO) and positive controls (astemizole; VRT-325; DMSO, 29°C) are shown. Arrows indicating core-glycosylated hERG G601S, and complex-glycosylated hERG G601S, which represents the corrected protein, are shown. Tubulin is shown as a loading control. (F) Densitometric quantitation of core and complex-glycosylated bands for selected treatments is shown. Error bars indicate SD (n = 3).

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172