Fig. 3

Western blots with nesprin-1 mAbs. Extracts of a cultured human dermal fibroblasts probed with the new mAbs against exons 81 to 86 of nesprin-1-giant and b normal adult human skeletal muscle probed with the new mAb N1G-Ex130 against exon 130 of nesprin-1-giant. The molecular weight marker used for blots (a) and (b) was: EZ-Run Pre-Stained Rec Protein Ladder (Fisher; Cat No: BP3603), upper molecular weight 170 kDa. Blot (c) is human skeletal muscle extract alongside Precision Plus Protein Standards (BioRad; Cat No: 161-0374) as molecular weight markers, upper molecular weight 250 kDa. Strips of blot (c) were probed with MANDRA1 to show dystrophin as a size marker (427 kDa). Bands with nesprin-1 mAbs were higher than the 427 kDa marker and consistent with nesprin-1-giant (1008 kDa). All blots include the C-terminal mAb against nesprin-1, MANNES1E [26]. * = likely non-specific bands; # = likely nesprin degradation products