Figure 7.

CFTR correctors rescued the sarcoglycan complex in LGMD2D myotubes. Myogenic cells from a patient carrying the L31P/V247M α-SG mutations were grown and differentiated for 7 days and treated for the last 48 h with 1‰ DMSO (vehicle) or the indicated CFTR correctors. At the end of incubation intact myotubes (not permeabilized) were labelled with antibodies recognizing an extracellular epitope of either α-SG (on the left) or δ-SG (on the right), as indicated, to mark the membrane resident sarcoglycans only. Primary antibodies were revealed with the secondary DyLight 488-conjugated anti-rabbit antibodies. Bars indicate 31.75 µm. Images were recorded with a Leica SP5 laser scanning confocal microscope at the same setting conditions.