Fig. 4.

LMNA patient-based cell models show nuclear deformities but no changes in glutathione levels. (A) iPS cell derived cardiomyocytes from patients were stained with DAPI and antibodies to lamin A/C and cardiac Troponin T (cTnT). The LMNA mutation producing lamin A/C G449V increased nuclear deformities (arrows) relative to controls expressing wild type lamin A/C. Yellow boxes indicate enlarged regions (right). Scale Bar: 30 μm. (B) Reduced and oxidized glutathione levels were measured in iPS cell derived cardiomyocytes. GSH and GSSG levels were measured in three to six independent biological samples. GSH was normalized to total glutathione and plotted as fluorescence intensity. Likewise, GSSG was normalized to total glutathione and plotted as fluorescence intensity. Unpaired Student's t-tests were then performed revealing no difference between patient-based cells and controls. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)