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Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4
Published on: August 21, 2017
Neuromyelitis optica-like pathology is dependent on type I interferon response
Reza Khorooshi1, Agnieszka Wlodarczyk, Nasrin Asgari
1Neurobiology, Institute of Molecular Medicine, University of Southern Denmark, Denmark.
Experimental Neurology
|February 26, 2013
Summary
Type I interferon signaling exacerbates neuromyelitis optica (NMO) lesions. Blocking this pathway in mice reduced damage, suggesting a therapeutic target for this autoimmune central nervous system disease.
Area of Science:
- Neuroimmunology
- Autoimmune diseases
- Central nervous system disorders
Background:
- Neuromyelitis optica (NMO) is an antibody-mediated autoimmune inflammatory disease affecting the central nervous system.
- Interferon beta, used for multiple sclerosis, may worsen NMO.
- The role of type I interferon in NMO pathogenesis is unclear.
Purpose of the Study:
- To investigate the role of type I interferon signaling in the formation of NMO lesions.
- To determine if blocking type I interferon signaling impacts NMO lesion development.
Main Methods:
- Intracerebral injection of patient-derived immunoglobulin G and human complement into wildtype and type I interferon receptor-deficient mice.
- Assessment of lesion formation and markers of neuronal damage (aquaporin-4, glial fibrillary acidic protein).
Main Results:
- Loss of aquaporin-4 and glial fibrillary acidic protein was significantly reduced in type I interferon receptor-deficient mice compared to wildtype.
- This indicates that type I interferon signaling is involved in the damage associated with NMO lesions.
Conclusions:
- Type I interferon signaling contributes to the pathogenesis of neuromyelitis optica.
- Targeting type I interferon pathways may be a potential therapeutic strategy for NMO.
Keywords:
AQP4Aquaporin-4AstrocytesCNSC′EAEGFAPH&EIFN alpha/beta receptorIFNARInflammationLFBMSNMONeuromyelitis opticaType I IFNaquaporin-4central nervous systemcomplementexperimental autoimmune encephalomyelitisglial fibrillary acidic proteinhematoxylin and eosinluxol fast bluemultiple sclerosisneuromyelitis optica
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