Recognition of desmoglein 3 by autoreactive T cells in pemphigus vulgaris patients and normals

M. Hertl, M. Amagai, H. Sundaram, J. Stanley, K. Ishii, S. I. Katz

研究成果: Chapter

105 被引用数 (Scopus)


Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and is caused by autoantibodies against desmoglein 3 (Dsg3) on epidermal keratinocytes. Because the production of autoantibodies is presumably T cell dependent, Dsg3-specific T cell reactivity was investigated in 14 PV patients and 12 healthy donors. Peripheral blood mononuclear cells from seven PV patients with active disease showed a primary in vitro response to a recombinant protein containing the extracellular portion (EC1-5) of Dsg3, whereas two of seven PV patients in remission or under immunosuppressive treatment exhibited only secondary (2°) or tertiary (3°) T cell responses to Dsg3. T cell responses to Dsg3 were also observed in four of 12 healthy individuals upon 2°or 3°stimulation with Dsg3. Both PV patients and healthy responders were either positive for DRβ1*0402 - which is highly prevalent in PV - or positive for DR11 alleles homologous to DRβ1*0402. Two CD4 + Dsg3-specific T cell lines and 12 T cell clones from two PV patients and two CD4 + T cell lines and eight T cell clones from two normals were also stimulated by a Dsg3 protein devoid of the EC2-3 (ΔN1), suggesting that epitopes were located in the EC1, EC4, and/or EC5. Using Dsg3 peptides, one immunodominant peptide (residues 161-177) was also identified in the EC2. These observations demonstrate that T cell responses to Dsg3 can be detected in PV patients and in healthy donors carrying major histocompatibility class II alleles identical or similar to those highly prevalent in PV.

ホスト出版物のタイトルJournal of Investigative Dermatology
出版社Nature Publishing Group
出版ステータスPublished - 1998

ASJC Scopus subject areas

  • 生化学
  • 分子生物学
  • 皮膚病学
  • 細胞生物学


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