Fig. 6 | Scientific Reports

Fig. 6

From: ABCB4 disease-causing variants S242R, S346I, T437I and T1077M significantly impair its function and display differential sensitivity to potentiators

Fig. 6

PC secretion activity of ABCB4-wt and the mutants and response to potentiators. HEK293 cells were transiently transfected with plasmids encoding ABCB4-wt or the indicated mutants, and PC secretion was measured after 24 h in the absence (-) or presence (+) of 10 µmol/L of ivacaftor (A) and 0,1 µmol/L of SBC219 (B). PC secretion was normalized to expression level of the mature form of the respective protein (ABCB4-wt or mutants) and expressed as a percentage of the secretion activity of ABCB4-wt. Results are means (± SD) of at least four independent experiments performed in triplicate. *p < 0.05; ** p < 0.01;***p < 0.001, **** p < 0.0001.

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