Desmogleine

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Desmogleins are a group of proteins that are built into the cell membrane and, as an integral membrane protein, are involved in cell-cell contacts .

Desmoglein belongs to the group of calcium- binding cell adhesion molecules , more precisely the desmosomal cadherins . As a transmembrane glycoprotein, it is an integral part of the desmosomes ( macula adhaerens ), which form an intercellular contact . It is found in vertebrates , mainly in cells of the epithelia and the heart muscle . Together with desmocollin , desmoglein is the essential adhesive protein in a desmosome and can be found integrated into the plasma membrane in condensation, so-called adhesion plaques . Facing the intercellular space, glycosylated parts of desmoglein proteins protrude into the intercellular gap ( desmoglea ), where they attach to other proteins in the neighboring cell and thus establish an intercellular connection. The connection between the desmoglein and the cytoplasmic intermediate filaments , which secures the intercellular contact , is mediated by desmoplakin , which is additionally strengthened by the plakoglobin in this connection.

There are four forms in the desmoglein family: 1, 2, 3 and 4, encoded by different genes that are all located on chromosome 18 in humans . The desmosomes of myocardial cells contain desmoglein-2 (DSG-2), in which multi-layered squamous epithelia are found mainly desmoglein-3, desmoglein-4 in highly differentiated keratinocytes of hair follicles , and desmoglein-1 in the upper layers of the epidermis .

Mutations in the genes coding for the desmogleins can be responsible for rare hereditary diseases, such as palmoplantar keratoderma (SPPK1, cause: DSG-1 ), arrhythmogenic right ventricular cardiomyopathy (ARCM, cause: DSG-2 ), and hypotrichosis (LAH1, cause : DSG-4 ). Autoantibodies against desmoglein-3 are the cause of pemphigus vulgaris ; in pemphigus foliaceus , however, desmoglein-1 is the autoantigen .

literature

Individual evidence

  1. see UniProt Q02413 (DSG1), UniProt Q14126 (DSG2), UniProt P32926 (DSG3), UniProt Q86SJ6 (DSG4).