Figure 6.

A pharmaceutical corrector rescued expression of α3 and enhanced its trafficking out of the endoplasmic reticulum.A, replicate representative blots from one experiment were cut and stained with different antibodies. Incubation in 4PBA for 48 h resulted in a relative increase in α3 and decrease in α1; no change in the total amount of β1; but a shift of β1 in L924P-expressing cells from the immature to the mature glycosylated form, a marker of transfer from endoplasmic reticulum to Golgi. B–D, the results were quantified for n = 3 or 4 independent experiments. Scans of lanes were individually normalized to actin or GAPDH. B, reciprocal changes in α3 and α1, normalized to the control untreated sample in each experiment. The reciprocal change in expression is consistent with increased competition of the exogenous α3 for a rate-limiting factor, such as β subunit. C, expression of β1 appeared to be increased slightly but not significantly by 4PBA in all cell lines. D, the proportions of immature β1 were the same as seen in Figure 1 in the three cell lines. For L924P only, there was a statistically significant reduction in the immature form, p < 0.01, with a shift to the mature form.