desmogleenindesmogleins

Desmoglein and desmogleins refer to a family of cadherin proteins that play a crucial role in the formation and maintenance of desmosomes, which are specialized cell-cell adhesion structures. These proteins are essential components of the epidermis and other stratified epithelial tissues, providing mechanical strength and integrity. There are four main desmoglein isoforms: desmoglein 1, desmoglein 2, desmoglein 3, and desmoglein 4.

Desmogleins are transmembrane proteins, with their extracellular domains mediating homophilic or heterophilic interactions with desmogleins on adjacent cells. This interaction is critical for establishing stable cell-cell junctions. The intracellular domains of desmogleins interact with other desmosomal proteins, such as plakoglobin and desmoplakin, to link the desmosome to the keratin intermediate filament cytoskeleton. This intricate network contributes significantly to the overall mechanical stability of tissues.

Dysfunction or mutations in desmogleins are implicated in various skin blistering diseases. For example, autoantibodies against desmoglein 1 are a hallmark of pemphigus foliaceus, leading to superficial blistering. Antibodies against desmoglein 3 are associated with pemphigus vulgaris, causing deeper blisters. Similarly, mutations in desmoglein 4 can result in inherited conditions like ectodermal dysplasia and hair defects. The precise role and interaction of each desmoglein isoform contribute to the specific characteristics and locations of these diseases, highlighting their vital importance in maintaining epithelial homeostasis.