Autoantibodies to type VII collagen recognize epitopes in a fibronectin-like region of the noncollagenous (NC1) domain
| Autor: | Karen M. Padilla, Phillip Prisayanh, Stephen W. Hunt, Mark W. Jenison, Dedee F. Murrell, W. Ray Gammon, Robert A. Briggaman, Drew A. Jones |
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| Rok vydání: | 1993 |
| Předmět: |
Epidermolysis bullosa acquisita
Pathology medicine.medical_specialty Recombinant Fusion Proteins Blotting Western Sequence Homology Enzyme-Linked Immunosorbent Assay Dermatology Epidermolysis Bullosa Acquisita Biochemistry Epitopes Dermis medicine Humans Lupus Erythematosus Systemic skin and connective tissue diseases Molecular Biology Autoantibodies Basement membrane integumentary system biology Chemistry Autoantibody Cell Biology DNA medicine.disease Connective tissue disease Fibronectins Fibronectin medicine.anatomical_structure Immunoglobulin G biology.protein Lamina densa Epidermolysis bullosa Collagen |
| Zdroj: | The Journal of investigative dermatology. 100(5) |
| ISSN: | 0022-202X |
| Popis: | Autoantibodies to type VII collagen are characteristic of the blistering diseases epidermolysis bullosa acquisita and bullous systemic lupus erythematosus (SLE). Blisters in those diseases are due to defective adhesion of the lamina densa subregion of the epithelial basement membrane to the underlying dermis. Previous studies indicating that type VII collagen contributes to lamina densa-dermal adhesion by cross-linking lamina densa and dermal matrix proteins suggests that autoantibodies may contribute to blisters by interfering with type VII collagen function. That hypothesis is supported by previous studies showing autoantibodies from a small number of epidermolysis bullosa acquisita patients recognize proteolytic fragments containing the 145- kD noncollagenous domain of type VII collagen. In this study, we examined reactivity of autoantibodies from a large number of epidermolysis bullosa acquisita and bullous SLE patients with fusion proteins representing most of the noncollagenous domain of type VII collagen and that those regions are homologous to type III repeats of fibronectin. These results suggest autoantibodies binding to fibronectin homology regions within the 145-kD noncollagenous domain may interfere with the adhesion function of type VII collagen and contribute to lamina densa-dermal dysadhesion in epidermolysis bullous acquisita and bullous SLE. |
| Databáze: | OpenAIRE |
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