Figure 2.
Schematics of the intercellular keratinocyte desmosome and the basal keratinocyte hemidesmosome at the basement membrane. Dsgs, the targets of pemphigus antibodies, mediate cell-cell contact by trans- and cis-adhesion. Adhesion is also provided by Dscs, which, like Dsgs, are in the cadherin adhesion molecule supergene family. Intracellular proteins of desmosomes, such as PG, PKP-1, and DP, link Dsgs and Dscs to KIFs. The hemidesmosome links basal keratinocytes to the epidermal basement membrane zone. BP180 and BP230 are the molecules targeted by autoantibodies in BP patients. BP230 and plectin link the hemidesmosome to KIFs. DP, BP230, and plectin, all of which link the KIFs to the desmosome or hemidesmosome, are in the same gene family of plakins. Abbreviations: BP, bullous pemphigoid; Col, collagen; DP, desmoplakin; Dsc, desmocollin; Dsg, desmoglein; KIF, keratin intermediate filament; Lam332, laminin 332; LD, lamina densa; LL, lamina lucida; PG, plakoglobin; PKP-1, plakophilin 1; SLD, sublamina densa.