Figure 3.

Representative images of αII-spectrin protein expression and staining pattern in fibroblast cells derived from 2 patients and unrelated controls. A. Western blot. 1. Western blotting of protein extracted from fibroblast cell lines of patients 1 and 29 and 3 wild-type age-matched controls. 2. Densitometric analysis of western blot using BioRad Image Lab software after relative normalization to actin as a housekeeping protein. The analysis showed no change in protein expression in patient 1 but showed a quantitative reduction of protein expression in patient 29. B. Immunocytochemical staining of αII-spectrin expression in primary fibroblasts of patients 1 and 29 and unrelated control individual with Alexa Fluor 488 conjugated secondary antibody (green) and Hoescht 33342 nuclear staining (blue). Scale bar represents 50 μm. Immunocytochemical staining showed high immunofluorescence brightness and intense immunoreactivity and aggregation of αII-spectrin in both studied patients compared with the healthy unrelated control. Ctr, control.