Desmoglein-1-specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem)

Citation
Ms. Lin et al., Desmoglein-1-specific T lymphocytes from patients with endemic pemphigus foliaceus (fogo selvagem), J CLIN INV, 105(2), 2000, pp. 207-213
Citations number
48
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Medical Research General Topics
Journal title
JOURNAL OF CLINICAL INVESTIGATION
ISSN journal
0021-9738 → ACNP
Volume
105
Issue
2
Year of publication
2000
Pages
207 - 213
Database
ISI
SICI code
0021-9738(200001)105:2<207:DTLFPW>2.0.ZU;2-P
Abstract
Pogo selvagem (FS), the endemic form of pemphigus foliaceus, is a cutaneous autoimmune disease characterized by subcorneal blistering of the epidermis and the production of autoantibodies against the desmosomal antigen desmog lein-1 (Dsg1). Previously, we showed that mice injected with autoantibodies from FS patients develop a skin disease that reproduces the clinical, hist ological, and immunological features of FS, indicating that autoantibodies play an essential role in the development of this disease. The purpose of t his study was to characterize the autoimmune T-cell response associated wit h FS. We provide here the first evidence, to our knowledge, that the great majority of FS patients have circulating T lymphocytes that specifically pr oliferate in response to the extracellular domain of Dsg1. Long-term T cell s developed from these patients also responded to Ds,ol, and this antigen-s pecific response was shown to be restricted to HLA-DR molecules. These Dsg1 -reactive FS T cells exhibited a CD4-positive memory T-cell phenotype and p roduced a T helper 2-like cytokine profile. These findings represent the in itial steps in defining the role of T cells in FS autoimmunity.