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. Author manuscript; available in PMC: 2010 Jan 29.
Published in final edited form as: J Invest Dermatol. 2009 Apr 2;129(9):2156. doi: 10.1038/jid.2009.61

Figure 9. Proteolytic processing of the precursor form of desmogleins (Dsgs) and mAb binding to the precursor and mature form of Dsg1.

Figure 9

(a) Dsgs are thought to be synthesized as inactive precursor Dsgs with prosequences in the endoplasmic reticulum. The prosequences are then removed by subtillisin-like proprotein convertases before transporting them to the plasma membrane and mature Dsgs are assembled into desmosomes. (b) Binding of anti-Dsg1 mAbs to the mature and the precursor form of Dsg1. The pathogenic mAb, 3–30/3h (shown in orange) binds to the mature Dsg1, but not the precursor Dsg1. Some non-pathogenic mAbs, 3–97/1c and 3–94/O18O8 (shown in green), bind to the precursor Dsg1. A non-pathogenic mAb, 1–18/L12 (shown in blue), which recognizes the middle portion of Dsg1, binds to both mature and precursor Dsg1. The pathogenic mAb bind the region of adhesive interface of Dsg1, which are unmasked by proteolytic processing, perhaps blocking trans-interaction of Dsgs.