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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
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Disease relevance of Pemphigus


High impact information on Pemphigus

  • We also show that DSG4 is an autoantigen in pemphigus vulgaris [6].
  • The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research [1].
  • Protection against pemphigus foliaceus by desmoglein 3 in neonates [7].
  • Fatal pemphigus vulgaris in a patient taking piroxicam [8].
  • Experiments exploiting the production of both pathogenic and nonpathogenic antidesmoglein antibodies in pemphigus patients' sera have afforded data that make an invaluable contribution towards identifying the functional domains of the desmogleins involved in intercellular adhesion [9].

Chemical compound and disease context of Pemphigus

  • Penicillamine-induced pemphigus [10].
  • Previous studies have shown that pemphigus vulgaris (PV) IgG will fix early complement components (C1q, C4, and C3) to cultured murine epidermal cell surfaces and that PV IgG and complement alter epidermal cell membrane integrity [11].
  • Pemphigus vulgaris antigen bound to the concanavalin A column and was released by 0.02 M methyl alpha-D-mannopyranoside [12].
  • This antigen was identified by transfer blotting to nitrocellulose paper, incubated with pemphigus vulgaris serum, or 20 control sera, and detected with fluorescein labeled antisera to human IgG [12].
  • Various techniques to extract the membrane-rich pellet from human epidermal homogenate were compared; 1% sodium dodecyl sulfate (SDS) and 1% dimethylsulfoxide proved to be superior to extract the pemphigus vulgaris antigen [12].

Biological context of Pemphigus

  • Serum from patients with pemphigus foliaceus or pemphigus vulgaris (but not from four normal controls) bound desmoglein and the 130-kd polypeptide, respectively, showing that these peptides (and not plakoglobin) are the antigenic binding sites in these disorders [13].
  • Complementary DNA cloning of the 130-kD pemphigus vulgaris (PV) autoantigen (PVA) has indicated that it is a member of the cadherin family of Ca(2+)-dependent cell adhesion molecules [14].
  • These results indicate that this baculovirus product has the proper conformation of the authentic PVA and that its conformation is important in pathogenicity of pemphigus [3].
  • Thus, the anti-desmoglein antibody profiles in pemphigus sera and the normal tissue distributions of Dsg1 and Dsg3 determine the sites of blister formation [15].
  • Induction of pemphigus phenotype by a mouse monoclonal antibody against the amino-terminal adhesive interface of desmoglein 3 [16].

Anatomical context of Pemphigus

  • Desmoglein-1-specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem) [5].
  • Using passive transfer of pemphigus IgG to normal and DSG3(null) neonatal mice, we show that in the areas of epidermis and mucous membrane that coexpress Dsg1 and Dsg3, antibodies against either desmoglein alone do not cause spontaneous blisters, but antibodies against both do [15].
  • In patients with pemphigus vulgaris (PV), autoantibodies against desmoglein 3 (Dsg3) cause loss of cell-cell adhesion of keratinocytes in the basal and immediate suprabasal layers of stratified squamous epithelia [17].
  • The pemphigus sera exhibit positive immunoblotting reactions with desmosome-enriched fractions obtained from bovine tongue epithelium [18].
  • Dsg1, the prototype of the desmoglein family, is a major component of epidermal desmosomes and the antigenic target of antibodies found in the sera of patients with the blistering disease, pemphigus foliaceus [19].

Gene context of Pemphigus

  • The notion that anti-Dsg antibodies alone are responsible for blisters in patients with pemphigus vulgaris (PV) stems from the ability of rDsg1 and rDsg3 to absorb antibodies that cause PV-like skin blisters in neonatal mice [20].
  • These findings suggest that Dsg4 may play a role other than adhesion and that the cross-reactivity of desmoglein autoantibodies should be factored into the framework of future studies of autoimmune mechanisms in pemphigus [21].
  • Pemphigus vulgaris antigen (desmoglein 3) is localized in the lower epidermis, the site of blister formation in patients [22].
  • These data localize Dsg3 exactly to the area in the epidermis where blisters occur in pemphigus vulgaris [22].
  • Another desmosomal cadherin, desmocollin, is occasionally recognized by certain pemphigus sera [23].

Analytical, diagnostic and therapeutic context of Pemphigus


  1. The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. Warren, S.J., Lin, M.S., Giudice, G.J., Hoffmann, R.G., Hans-Filho, G., Aoki, V., Rivitti, E.A., Santos, V., Diaz, L.A. N. Engl. J. Med. (2000) [Pubmed]
  2. A molecular basis for MHC class II--associated autoimmunity. Todd, J.A., Acha-Orbea, H., Bell, J.I., Chao, N., Fronek, Z., Jacob, C.O., McDermott, M., Sinha, A.A., Timmerman, L., Steinman, L. Science (1988) [Pubmed]
  3. Absorption of pathogenic autoantibodies by the extracellular domain of pemphigus vulgaris antigen (Dsg3) produced by baculovirus. Amagai, M., Hashimoto, T., Shimizu, N., Nishikawa, T. J. Clin. Invest. (1994) [Pubmed]
  4. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. Amagai, M., Nishikawa, T., Nousari, H.C., Anhalt, G.J., Hashimoto, T. J. Clin. Invest. (1998) [Pubmed]
  5. Desmoglein-1-specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem). Lin, M.S., Fu, C.L., Aoki, V., Hans-Filho, G., Rivitti, E.A., Moraes, J.R., Moraes, M.E., Lazaro, A.M., Giudice, G.J., Stastny, P., Diaz, L.A. J. Clin. Invest. (2000) [Pubmed]
  6. Desmoglein 4 in hair follicle differentiation and epidermal adhesion: evidence from inherited hypotrichosis and acquired pemphigus vulgaris. Kljuic, A., Bazzi, H., Sundberg, J.P., Martinez-Mir, A., O'Shaughnessy, R., Mahoney, M.G., Levy, M., Montagutelli, X., Ahmad, W., Aita, V.M., Gordon, D., Uitto, J., Whiting, D., Ott, J., Fischer, S., Gilliam, T.C., Jahoda, C.A., Morris, R.J., Panteleyev, A.A., Nguyen, V.T., Christiano, A.M. Cell (2003) [Pubmed]
  7. Protection against pemphigus foliaceus by desmoglein 3 in neonates. Wu, H., Wang, Z.H., Yan, A., Lyle, S., Fakharzadeh, S., Wahl, J.K., Wheelock, M.J., Ishikawa, H., Uitto, J., Amagai, M., Stanley, J.R. N. Engl. J. Med. (2000) [Pubmed]
  8. Fatal pemphigus vulgaris in a patient taking piroxicam. Martin, R.L., McSweeney, G.W., Schneider, J. N. Engl. J. Med. (1983) [Pubmed]
  9. Desmosomes and disease: pemphigus and bullous impetigo. Payne, A.S., Hanakawa, Y., Amagai, M., Stanley, J.R. Curr. Opin. Cell Biol. (2004) [Pubmed]
  10. Penicillamine-induced pemphigus. Zone, J., Ward, J., Boyce, E., Schupbach, C. JAMA (1982) [Pubmed]
  11. Complement fixation by pemphigus antibody. V. Assembly of the membrane attack complex on cultured human keratinocytes. Xia, P., Jordon, R.E., Geoghegan, W.D. J. Clin. Invest. (1988) [Pubmed]
  12. Isolation and purification of a pemphigus vulgaris antigen from human epidermis. Peterson, L.L., Wuepper, K.D. J. Clin. Invest. (1984) [Pubmed]
  13. Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliaceus and pemphigus vulgaris. Korman, N.J., Eyre, R.W., Klaus-Kovtun, V., Stanley, J.R. N. Engl. J. Med. (1989) [Pubmed]
  14. Autoantibodies against the amino-terminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic. Amagai, M., Karpati, S., Prussick, R., Klaus-Kovtun, V., Stanley, J.R. J. Clin. Invest. (1992) [Pubmed]
  15. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. Mahoney, M.G., Wang, Z., Rothenberger, K., Koch, P.J., Amagai, M., Stanley, J.R. J. Clin. Invest. (1999) [Pubmed]
  16. Induction of pemphigus phenotype by a mouse monoclonal antibody against the amino-terminal adhesive interface of desmoglein 3. Tsunoda, K., Ota, T., Aoki, M., Yamada, T., Nagai, T., Nakagawa, T., Koyasu, S., Nishikawa, T., Amagai, M. J. Immunol. (2003) [Pubmed]
  17. Targeted disruption of the pemphigus vulgaris antigen (desmoglein 3) gene in mice causes loss of keratinocyte cell adhesion with a phenotype similar to pemphigus vulgaris. Koch, P.J., Mahoney, M.G., Ishikawa, H., Pulkkinen, L., Uitto, J., Shultz, L., Murphy, G.F., Whitaker-Menezes, D., Stanley, J.R. J. Cell Biol. (1997) [Pubmed]
  18. Further analysis of pemphigus autoantibodies and their use in studies on the heterogeneity, structure, and function of desmosomes. Jones, J.C., Yokoo, K.M., Goldman, R.D. J. Cell Biol. (1986) [Pubmed]
  19. Structure of DSG1, the bovine desmosomal cadherin gene encoding the pemphigus foliaceus antigen. Evidence of polymorphism. Puttagunta, S., Mathur, M., Cowin, P. J. Biol. Chem. (1994) [Pubmed]
  20. Antibodies against keratinocyte antigens other than desmogleins 1 and 3 can induce pemphigus vulgaris-like lesions. Nguyen, V.T., Ndoye, A., Shultz, L.D., Pittelkow, M.R., Grando, S.A. J. Clin. Invest. (2000) [Pubmed]
  21. Defining the pathogenic involvement of desmoglein 4 in pemphigus and staphylococcal scalded skin syndrome. Nagasaka, T., Nishifuji, K., Ota, T., Whittock, N.V., Amagai, M. J. Clin. Invest. (2004) [Pubmed]
  22. Pemphigus vulgaris antigen (desmoglein 3) is localized in the lower epidermis, the site of blister formation in patients. Amagai, M., Koch, P.J., Nishikawa, T., Stanley, J.R. J. Invest. Dermatol. (1996) [Pubmed]
  23. A case of pemphigus vulgaris showing reactivity with pemphigus antigens (Dsg1 and Dsg3) and desmocollins. Hashimoto, T., Amagai, M., Watanabe, K., Dmochowski, M., Chidgey, M.A., Yue, K.K., Garrod, D.R., Nishikawa, T. J. Invest. Dermatol. (1995) [Pubmed]
  24. Human autoantibodies against desmosomes: possible causative factors in pemphigus. Jones, J.C., Arnn, J., Staehelin, L.A., Goldman, R.D. Proc. Natl. Acad. Sci. U.S.A. (1984) [Pubmed]
  25. Restoration of the epidermal phenotype by follicular outer root sheath cells in recombinant culture with dermal fibroblasts. Limat, A., Breitkreutz, D., Hunziker, T., Boillat, C., Wiesmann, U., Klein, E., Noser, F., Fusenig, N.E. Exp. Cell Res. (1991) [Pubmed]
  26. Use of domain-swapped molecules for conformational epitope mapping of desmoglein 3 in pemphigus vulgaris. Futei, Y., Amagai, M., Sekiguchi, M., Nishifuji, K., Fujii, Y., Nishikawa, T. J. Invest. Dermatol. (2000) [Pubmed]
  27. Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus. Amagai, M., Hashimoto, T., Green, K.J., Shimizu, N., Nishikawa, T. J. Invest. Dermatol. (1995) [Pubmed]
  28. Effects of steroid, retinoid, and protease inhibitors on the formation of acantholysis induced in organ culture of skins from patients with benign familial chronic pemphigus. Ikeda, S., Ogawa, H. J. Invest. Dermatol. (1991) [Pubmed]
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