Scientific background: |
Desmoglein3 is also known as PVA(Pemphigus Vulgaris Antigen). Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune diseases of the skin which have as target antigens 2 different members of the desmoglein subfamily of the desmosomal cadherins: pemphigus vulgaris antigen (PVA, or desmoglein-3) in the case of PV and desmoglein I in the case of PF . The DSG3 gene, like the genes for DSG1 and DSG2 , is located on chromosome 18. The human DSG3 gene consists of 15 exons and spans more than 23 kb. DSG3 and DSG1 are the autoantigens of pemphigus vulgaris and pemphigus foliaceus, respectively. DSG3 and DSG1 are the target antigens in PNP, and IgG autoantibodies against DSG3 in PNP sera play a pathogenic role in the loss of keratinocyte adhesion and blistering. |
References: |
1.Amagai, M., Klaus-Kovtun, V., Stanley, J. R.Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion.Cell 67: 869-877, 1991.
2.Arnemann, J., Spurr, N. K., Buxton, R. S.The human gene (DSG3) coding for the pemphigus vulgaris antigen is, like the genes coding for the other two known desmogleins, assigned to chromosome 18.Hum. Genet. 89: 347-350, 1992.
3.Silos, S. A., Tamai, K., Li, K., Kivirikko, S., Kouba, D., Christiano, A. M., Uitto, J.Cloning of the gene for human pemphigus vulgaris antigen (desmoglein 3), a desmosomal cadherin.J. Biol. Chem. 271: 17504-17511, 1996.
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