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Blood-brain barrier dysfunction in immuno-mediated neurological diseases
Fumitaka Shimizu1, Hideaki Nishihara1, Takashi Kanda1
1a Department of Neurology and Clinical Neuroscience , Yamaguchi University Graduate School of Medicine , Ube , Japan.
Immunological Medicine
|April 3, 2019
Summary
The blood-brain barrier (BBB) is crucial for brain health. Dysfunction in BBB integrity is implicated in neurological diseases like MS and NMO, with specific autoantibodies contributing to BBB breakdown.
Area of Science:
- Neuroimmunology
- Neurology
- Vascular Biology
Background:
- The blood-brain barrier (BBB) protects the brain and its dysfunction is key in immuno-mediated neurological diseases.
- Diseases like multiple sclerosis (MS), neuromyelitis optica (NMO), and neuropsychiatric systemic lupus erythematosus (NPSLE) exhibit BBB breakdown in active lesions.
Purpose of the Study:
- To review the structure and function of the BBB.
- To discuss the pathophysiology of BBB breakdown in neurological diseases.
- To present recent findings on autoantibody-mediated BBB breakdown.
Main Methods:
- Review of existing literature on BBB structure, function, and disease pathology.
- Analysis of pathological findings in patients with SPMS, NMO, and NPSLE.
- Identification and characterization of novel BBB-reactive autoantibodies.
Main Results:
- BBB dysfunction is a critical factor in immuno-mediated neurological diseases.
- NMO involves pathogenic aquaporin 4 (AQP4) autoantibodies crossing the BBB.
- NPSLE is associated with BBB disruption and endothelial cell-binding antibodies.
- Galectin-3 and GRP78 autoantibodies were identified as BBB-reactive in SPMS and NMO, respectively.
Conclusions:
- Specific autoantibodies play a role in BBB breakdown in neurological disorders.
- Understanding autoantibody-mediated BBB damage is crucial for disease pathogenesis.
- Further research into these autoantibodies may reveal new therapeutic targets.
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