title: Structure and function of desmosomal proteins and their role in development and disease.
abstract: Desmosomes represent major intercellular adhesive junctions at basolateral membranes of epithelial cells and in other tissues. They mediate direct cell-cell contacts and provide anchorage sites for intermediate filaments important for the maintenance of tissue architecture. There is increasing evidence now that desmosomes in addition to a simple structural function have new roles in tissue morphogenesis and differentiation. Transmembrane glycoproteins of the cadherin superfamily of Ca(2+)-dependent cell-cell adhesion molecules which mediate direct intercellular interactions in desmosomes appear to be of central importance in this respect. The complex network of proteins forming the desmosomal plaque associated with the cytoplasmic domain of the desmosomal cadherins, however, is also involved in junction assembly and regulation of adhesive strength. This review summarizes the structural features of these desmosomal proteins, their function during desmosome assembly and maintenance, and their role in development and disease.
authors.affiliation: Institute of Clinical Chemistry and Pathobiochemistry, Benjamin Franklin University Hospital, Hindenburgdamm 30, 12200 Berlin, Germany. otmar.huber@ukbf.fu-berlin.de
authors.forename: O
authors.initials: O
authors.lastname: Huber
mesh_terms: D020816:Amino Acid Motifs; D000818:Animals; D015820:Cadherins / Q000235:genetics / Q000378:metabolism; D002448:Cell Adhesion / Q000502:physiology; D002454:Cell Differentiation / Q000502:physiology; D003598:Cytoskeletal Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D003896:Desmosomes / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D007381:Intermediate Filament Proteins / Q000378:metabolism; D009024:Morphogenesis; D051190:Plectin; D012871:Skin Diseases / Q000235:genetics
chemical_list: D015820:Cadherins; D003598:Cytoskeletal Proteins; D007381:Intermediate Filament Proteins; C495332:PLEC protein, human; D051190:Plectin