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First published online February 22, 2006
doi: 10.1242/10.1242/jcs.02888

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The desmosome: cell science lessons from human diseases

¹ Department of Dermatology, Emory University School of Medicine, Emory University, Atlanta, GA 30322, USA
² Department of Cell Biology, Emory University School of Medicine, Emory University, Atlanta, GA 30322, USA

View larger version (93K): [in a new window]   Fig. 1. Electron micrograph and schematic representation of the desmosome. The desmosome is an electron-dense complex (upper panel) found in tissues subjected to mechanical stress, such as stratified squamous epithelia cells and the myocardium. This intercellular junction is composed of a core region, which mediates tight cell-cell adhesion, and a plaque region, which mediates attachment to the intermediate filament cytoskeleton. The core region contains the extracellular domains of the desmosomal cadherins, the desmocollins and desmogleins. The cytoplasmic plaque region includes the C-terminal tails of the desmosomal cadherins, which associate directly and indirectly with various cytoplasmic proteins. The armadillo family proteins in the desmosome include plakoglobin and plakophilins. These proteins mediate interactions between the desmosomal cadherin tails and desmoplakin, a plakin family protein that binds directly to intermediate filaments. These components of the desmosome allow tethering of the intermediate filaments to the plasma membrane, thereby acting as a scaffold to provide structural integrity to cells and tissues. KIF, keratin intermediate filaments.

View larger version (41K): [in a new window]   Fig. 2. Expression pattern of desmosomal components in the epidermis. Keratin filaments are shown connecting to desmosomes at sites of cell-cell contact and to hemidesmosomes at the basement membrane. The profiles and relative expression levels of various desmosomal proteins in the epidermal layers are depicted on the right.

View larger version (71K): [in a new window]   Fig. 3. Clinical appearance and pathohistology of various human desmosomal disorders. Pemphigus vulgaris (A and B) is characterized by the loss of intercellular adhesion between basal and suprabasal keratinocytes (A) and by skin blistering and erosions (B). The hallmark of palmoplantar keratoderma (C and D) is massive thickening of the stratum corneum (C), resulting in dramatically thickened skin on palms and soles (D). Arrhythmogenic right-ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement (*) of the myocardium (E). Please refer to Table 1 for additional information on specific molecular targets of each disease. Panel D is reprinted from (Rickman et al., 1999) with permission from Oxford University Press. Panel E is reprinted from (Protonotarios and Tsatsopoulou, 2004) with permission from Elsevier.

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