Expression of pemphigus-autoantigen desmoglein 1 in human thymus

Tissue Antigens. 2008 May;71(5):464-70. doi: 10.1111/j.1399-0039.2008.01020.x. Epub 2008 Mar 10.

Abstract

Desmoglein (Dsg) 1 is a transmembrane glycoprotein of the desmosome allowing cell-cell adhesion between keratinocytes, whose expression is restricted to stratified squamous epithelia-like epidermis. Dsg1 is the target autoantigen of pathogenic autoantibodies produced by pemphigus foliaceus and 50% of pemphigus vulgaris patients in a Dsg1-specific T-cell-dependent pathway. Herewith, we show that mRNA of the DSG1 gene is present in normal human thymus and show by quantitative real-time polymerase chain reaction analysis that the expression of DSG1 transcript increases with age. Although immunoblot analysis on human thymus extracts using different anti-Dsg1 antibodies did not allow to detect the protein, we show by double-immunofluorescence assay that Dsg1 is expressed at protein level by CD19+ CD63+ cells located in the medulla. These data provide another illustration of the thymic expression of a tissue-specific autoantigen involved in an organ-specific autoimmune disease, which may participate in the tolerance acquisition and/or regulation of Dsg1-specific T cells.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aging
  • Autoantigens / immunology
  • Autoantigens / metabolism*
  • Autoimmunity
  • Child
  • Child, Preschool
  • Desmoglein 1 / genetics
  • Desmoglein 1 / immunology
  • Desmoglein 1 / metabolism*
  • Epidermal Cells
  • Epidermis / immunology
  • Epidermis / metabolism
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Keratinocytes / cytology
  • Keratinocytes / immunology
  • Keratinocytes / metabolism
  • Male
  • Middle Aged
  • Pemphigus / genetics
  • Pemphigus / immunology*
  • Pemphigus / metabolism
  • Thymus Gland / cytology
  • Thymus Gland / immunology*
  • Thymus Gland / metabolism

Substances

  • Autoantigens
  • Desmoglein 1