DSG1

DSG1

A gene on chromosome 18q12.1 that encodes desmoglein 1, a calcium-binding transmembrane glycoprotein component of desmosomes in epithelial cells, which belongs to the cadherin cell adhesion molecule superfamily.

Molecular pathology
Desmoglein 1 is the antigen targeted in the autoimmune blistering disease pemphigus foliaceus; defects in DSG1 cause palmoplantar keratoderma striate type 1.
References in periodicals archive ?
Inmunoadsorcion: por medio de esta estrategia terapeutica se puede lograr la eliminacion rapida de autoanticuerpos circulantes contra Dsg1 y Dsg3.
It is mediated by pathogenic auto-antibodies (-Abs) that bind to desmoglein 1 (Dsg1) and lead to acantholysis and consequent blister formation in the subcorneal layer of the epidermis [2-4].
Mucocutaneous manifestations are the result of intercellular autoantibodies mainly of the IgG type working against desmosomal antigens desmoglein 3 (Dsg3) in the mucosal dominant type and additionally against desmoglein 1 (Dsg1) in the mucocutaneous type (20).
However, autoantibodies against various targets can be found, including desmosomal proteins Dsg1 and Dsg3, plakin family proteins (periplakin, envoplakin, plectin, desmoplakin, and BP180), and the protease inhibitor alpha-2-macroglobulin-like antigen 1 (A2ML1) (3, 20, 23).
The currently known target antigens are desmoglein 1 (Dsg1), desmoglein 3 (Dsg3), and plakin protein, which act as adhesion molecules between keratinocytes in the epidermis.
Desmogleins are calcium-binding transmembrane glycoproteins, members of the desmosomal cadherins that provide adhesive integrity to desmosomes between adjoining keratinocytes; They consist of proteins Dsg1, Dsg2, Dsg3, and Dsg4.
Blisters occur in the epidermis and the mucous membranes, where the IgG autoantibodies target two structural proteins of the desmosomes identified as desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3).A new pemphigus antigen, desmoglein 4, has recently been discovered and implicated in the pathogenesis of PV.