Figure 3.

Representative molecules at the nerve terminal and the agrin-LPR4-MuSK signaling pathway to induce AChR clustering. Interactions between binding domains are indicated by double headed arrows [71,98,100,101,102]. Broken arrows in the muscle indicate that the exact signaling molecules are not shown. Diseases other than CMS and toxins affecting the NMJ are indicated in red letters. βCAT, β-catenin; BPD, β-propeller domain; C6, six-cysteine-box; Ctgf, connective tissue growth factor; Fz-CRD, frizzled-like cysteine-rich domain; Ig, immunoglobulin-like domain; LDLR-A, low-density lipoprotein receptor class A repeat; Lgr5, leucine-rich repeat-containing G-protein coupled receptor 5; and Rspo2, R-spondin 2.