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Ocular cicatricial pemphigoid antigen: partial sequence and biochemical characterization
1Center for Blood Research, Harvard Medical School, Boston, MA 02115, USA.
Summary
Ocular cicatricial pemphigoid (OCP) is an autoimmune disease targeting beta 4-integrin. This finding helps classify OCP and understand epithelial attachment in autoimmune blistering diseases.
Area of Science:
- Immunodermatology
- Molecular Biology
- Autoimmunity
Background:
- Ocular cicatricial pemphigoid (OCP) is an autoimmune disease affecting squamous epithelia, characterized by epithelial separation at the basement membrane zone.
- Immunopathological studies show deposition of anti-basement membrane zone autoantibodies in OCP patients.
Purpose of the Study:
- To identify the autoantigens targeted in OCP.
- To elucidate the role of specific proteins in OCP pathogenesis and classification.
Main Methods:
- Screening of a human keratinocyte cDNA library with IgG from OCP patient sera.
- Identification of the targeted protein using Western blotting and antibody cross-reactivity.
- Comparison with sera from patients with other autoimmune blistering diseases.
Main Results:
- A cDNA clone homologous to the cytoplasmic domain of beta 4-integrin was identified.
- OCP sera recognized a 205-kDa protein identified as beta 4-integrin.
- Sera from other blistering diseases did not recognize this protein, confirming specificity.
Conclusions:
- Beta 4-integrin is strongly implicated in the pathogenesis of OCP.
- Molecular identification of autoantigens like beta 4-integrin aids in classifying OCP subsets.
- Understanding beta 4-integrin's role is crucial for epithelial attachment in autoimmune diseases.