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Pathogenicity of Misfolded and Dimeric HLA-B27 Molecules
Antony N Antoniou1, Izabela Lenart, David B Guiliano
1Division of Infection and Immunity/Centre of Rheumatology, Department of Immunology and Molecular Pathology, University College London, Windeyer Institute of Medical Science, 46 Cleveland Street, London W1T 4JF, UK.
International Journal of Rheumatology
|May 7, 2011
Summary
The strong link between HLA-B27 and spondyloarthropathies (SpAs) like ankylosing spondylitis may stem from HLA-B27 misfolding. This misfolding could trigger cellular events leading to chronic inflammation and disease progression in SpAs.
Area of Science:
- Immunogenetics
- Rheumatology
- Molecular Biology
Background:
- The human leukocyte antigen B27 (HLA-B27) is strongly associated with spondyloarthropathies (SpAs), a group of inflammatory arthritic diseases.
- This association is the most significant known between any HLA molecule and autoimmune disease.
- The underlying mechanism connecting HLA-B27 to SpA pathogenesis remains unclear.
Purpose of the Study:
- To explore the hypothesis that HLA-B27 misfolding is central to SpA development.
- To discuss the role of unusual HLA-B27 biochemical structures in SpA pathogenesis.
- To elucidate how these structures contribute to chronic inflammation and disease progression.
Main Methods:
- Review of existing animal model data.
- Analysis of biochemical data concerning HLA-B27 structure.
- Discussion of cellular events implicated in SpA.
Main Results:
- Biochemical and animal model data suggest HLA-B27 misfolding is a key factor.
- Unusual biochemical structures of HLA-B27 are implicated in SpA pathogenesis.
- These structures may drive cellular events leading to inflammation.
Conclusions:
- HLA-B27 misfolding is a plausible mechanism linking the gene to SpA.
- The unique biochemical properties of HLA-B27 are critical for disease development.
- Understanding these structures offers insights into chronic inflammation in SpAs.
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