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Table 1 F508del-CFTR corrector compounds show distinct profiles of correction for other ER-retained proteins

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

Corrector

CFTR F508del

hERG G601S

hERG F805C

SUR1 A116P

SUR1 V187D

V2R L292P

V2R V206D

VRT-325

+

+

-

-

ND

-

ND

Glycerol

+

ND

ND

+

ND

ND

+/−

29°C

++

+

+

+

+

++

++

KM60

+

+

-

+

+

-

+

KM57

+/−

++

-

+/−

-

-

+/−

ABT-888

+

-

ND

+

+

-

+

Glafenine

+

+

-

+

-

-

-

RDR1

+

-

-

+

+

-

-

Ouabain

+

+/−*

+*

+

+

+

+

Carbamazepine

+

-

ND

-

+/−

-

ND

Latonduine

+

+/−

-

+

++

+/−

+

Astemizole

ND

+

-

ND

ND

ND

ND

Glibenclamide

ND

ND

ND

++

++

ND

ND

  1. A qualitative assessment of correction as determined by glycosylation status in immunoblotting is shown for each mutation following treatment with a corrector compound, where “-“ indicates no correction observed, “+/−“ indicates slight correction, “+” and “++” indicate more and best correction observed, respectively, and “ND” indicates not determined. Asterisks indicate that an intermediate glycoform was observed, but no correction. Astemizole and glibenclamide were included as positive controls for correction of hERG G601S and SUR1 mutants, respectively.