Figure 1.

Dystrophin Exon Map, Downstream Exon 55, and the Dystrophin Glycoprotein Complex

(A) The majority of exons downstream of dystrophin exon 55 are out-of-frame. These exons encode important domains for the assembly of the dystrophin glycoprotein complex. SLR, spectrin-like repeats. (B) The distal third of dystrophin interacts, either directly or indirectly with proteins in the dystrophin glycoprotein complex, including α/β dystroglycans, α/β/γ/δ/ε sarcroglycans, α/β syntrophins, and α/β dystrobrevins to stabilize the sarcolemma. ABD, actin binding domain; H, hinge; DYG, dystroglycan; SYB, syntrophin binding site; CC1 and CC2, coiled coil 1 and 2 (image not to scale).