Search results for "Desmoglein 3"
showing 4 items of 4 documents
Langerhans Cells Prevent Autoimmunity via Expansion of Keratinocyte Antigen-Specific Regulatory T Cells
2017
Langerhans cells (LCs) are antigen-presenting cells in the epidermis whose roles in antigen-specific immune regulation remain incompletely understood. Desmoglein 3 (Dsg3) is a keratinocyte cell-cell adhesion molecule critical for epidermal integrity and an autoantigen in the autoimmune blistering disease pemphigus. Although antibody-mediated disease mechanisms in pemphigus are extensively characterized, the T cell aspect of this autoimmune disease still remains poorly understood. Herein, we utilized a mouse model of CD4+ T cell-mediated autoimmunity against Dsg3 to show that acquisition of Dsg3 and subsequent presentation to T cells by LCs depended on the C-type lectin langerin. The lack of…
Cleavage of desmoglein 3 can explain its depletion from keratinocytes in pemphigus vulgaris.
2008
We have previously demonstrated that serum of patients with pemphigus vulgaris induces reduction of desmoglein 3 (Dsg3) half-life in keratinocytes (FEBS Lett 2006: 580: 3276). This phenomenon seems to occur as a consequence of the progressive depletion of Dsg3 from desmosomes. Here we reported that reduction of full-length Dsg3 may be due to its progressive cleavage, leading to the formation of two fragmentation products with apparent molecular masses of about 60 kDa (fragment 1) and 70 kDa (fragment 2), as revealed by Western blotting. Unexpectedly, analysis of fragmentation pattern suggested cleavage to occur intracellularly. Consistently, fragment 1 was shed and localized within the cyto…
Evaluation of desmoglein 1 and 3 autoantibodies in pemphigus vulgaris: correlation with disease severity
2020
Background Pemphigus is an autoimmune blistering disease of the skin and mucous membranes caused by autoantibodies against desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3). Pemphigus vulgaris (PV) is the most common form of pemphigus. The aim of this study was to assess the correlation between the levels of anti-desmoglein 1 and 3 autoantibodies and the severity of PV disease. Material and Methods Nineteen newly diagnosed patients with pemphigus vulgaris were enrolled in this study. The titers of Dsg in subjects by using enzyme-linked immunosorbent assay (ELISA) were done at diagnosis time-point, 4th and 8th weeks after the initiation of treatment, and the correlation of antibodies with the oral…
Fallberichte
2004
High-dose immunoglobulins (IVIG) are important agents in the treatment of numerous autoimmune disorders. In the field of dermatology, increasing numbers of patients with bullous autoimmune disorders and mixed connective tissue diseases have been treated. We successfully treated a patient with pemphigus vulgaris with IVIG, after he had developed osteonecrosis of his right femoral head while on azathioprine and corticosteroids. After reduction of oral corticosteroids, his disease flared dramatically. The addition of a high dose immunosuppressive regimen including azathioprine and dapsone failed to help. Thus high dose IVIG (2 mg/kg, 4 weeks interval) therapy was initiated and induced complete…