Figure 1. Full-length and representative truncated, Becker MD dystrophin in the dystrophin-glycoprotein complex.

Schematic of dystrophin and the dystroglycan complex (DGC). In normal skeletal muscle, the muscle cytoskeleton (actin) is linked by dystrophin to extracellular matrix via the cell membrane. Boxed inset illustrates crystal structure for tandem repeats 2–3 from actinin with triple helix repeats linked by a continuous and extended helix. (A) Wild-type and (B) truncated dystrophin corresponding to milder BMD phenotype with in-frame exon 17–40 deletion.