[Cell adhesion molecules and extracellular matrix components as target structures of autoimmunity].
A number of cell-cell and cell-matrix adhesion molecules as well as several extracellular matrix components represent target structures of antibody-mediated autoimmunity which recently have been extensively characterized at the molecular biological level. Pathogenic autoantibodies against these molecules have been found to be causally related to disturbances of cell and tissue adhesion that become apparent as various (muco-)cutaneous blistering diseases. In desmosomes, desmosomal cadherins (desmogleins and desmocollins) mediate epidermal intercellular adhesion. Among these, desmoglein 1 and desmoglein 3 are the autoantigens of pemphigus foliaceus and pemphigus vulgaris, respectively, being characterized by intraepidermal blistering. The pathogenic relevance of autoantibodies against desmocollins (IgA pemphigus and other pemphigus types) and desmoplakins (paraneoplastic pemphigus) still remains unclear. Hemidesmosomes contain the plaque protein BPAG1 and the partly collagen-like transmembrane protein BPAG2, representing the autoantigens of bullous pemphigoid and pemphigoid gestations which show subepidermal blistering. A certain subtype of cicatricial (benign mucous membrane) pemphigoid is characterized by autoantibodies against laminin 5 present in the subhemidesmosomal anchoring filaments. Both epidermolysis bullosa acquisita and bullous SLE exhibit autoantibodies against collagen type VII which constitutes the anchoring fibrils. Besides, autoantibodies against a particular collagen type IV chain of the glomerular basement membrane are responsible for the manifestation of Goodpasture's syndrome. These recent molecular biological findings might be the basis for the development of novel therapeutic strategies.
Moll
R
R
Institut für Pathologie, Martin-Luther-Universität Halle-Wittenberg, Saale.
Bahn
H
H
Bayerl
C
C
Moll
I
I
D000818:Animals; D001323:Autoantibodies; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology*; D015551:Autoimmunity; D015815:Cell Adhesion Molecules / Q000276:immunology*; D003896:Desmosomes / Q000276:immunology / Q000473:pathology; D005109:Extracellular Matrix / Q000276:immunology; D016326:Extracellular Matrix Proteins / Q000276:immunology*; D006801:Humans; D010392:Pemphigus / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology
D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D016326:Extracellular Matrix Proteins
Zelluläre Adhäsionsmoleküle und Komponenten der extrazellulären Matrix als Zielstrukturen der Autoimmunität.