DSG3
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Desmoglein-3 (DSG3) is a member of the family of desmogleins (DSGs), as DSG1 (desmoglein-1).
Pathology
DSG3 is an autoantigen in pemphigus vulgaris.
Desmoglein-3 (DSG3) can be inactivated by IgG auto-antibodies causing pemphigus vulgaris. This autoantibody can be detected by direct and indirect diagnostic immunofluorescence testing of skin and serum, respectively. Lesional sites show a characteristic netlike pattern of intercellular IgG deposits localized to sites of developed or incipient acantholysis.
It is now known that the antibody in pemphigus vulgaris reacts with desmoglein-3 (DSG3), a component of the desmosomes that appear to bind keratinocytes together.
When the gene for desmoglein-3 (DSG-3) is disrupted in genetically engineered mice, suprabasal blisters akin to pemphigus develop owing to lack of desmosome adhesion.
This suggests a direct role for pemphigus autoantibodies in interfering with the function of this protein. Some of the acantholytic process may also be the consequence of synthesis and liberation of a serine protease (plasminogen activator) by epidermal cells, an event that is triggered by the pemphigus antibody.